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Page 1: Ensuring that all paediatricians and rheumatologists recognise significant rheumatic diseases

Best Practice & Research Clinical Rheumatology 23 (2009) 625–642

Contents lists available at ScienceDirect

Best Practice & Research ClinicalRheumatology

journal homepage: www.elsevierheal th.com/berh

4

Ensuring that all paediatricians and rheumatologistsrecognise significant rheumatic diseases

Helen Foster, MD, FRCP, FRCPCH, Professor Paediatric Rheumatology a,*,Yukiko Kimura, MD, Chief, Pediatric Rheumatology b,1

a Newcastle University, Medical School, Framlington Place, Newcastle upon Tyne, NE2 4HH, UKb Joseph M. Sanzari Children’s Hospital, Hackensack University Medical Center, 30 Prospect Avenue, Hackensack, NJ 07601, USA

Keywords:educationclinical skillscurriculumaccess to care

* Corresponding author. Tel.: þ44 191 2227541;E-mail addresses: [email protected] (H. Foste

1 Tel: þ1 201 996 5306; Fax: þ1 201 996 9815.

1521-6942/$ – see front matter � 2009 Elsevier Ldoi:10.1016/j.berh.2009.07.002

In many health-care systems around the world, many generalists(defined here as a clinician who is not an expert in paediatricmusculoskeletal [pMSK] medicine) will be the doctor to whomchildren with MSK problems are most likely to present. Thegeneralist is likely to have a clinical background in family medicineand paediatrics, but may be a specialist in adult orthopaedics, adultrheumatology or emergency care. Therefore, it is critical that thegeneralist is able to quickly recognise a child with significantrheumatic disease in order to initiate the proper treatment orreferral to a specialist. However, most generalists are ill equippedto be able to do so, because of current deficiencies in educationand training. The aims of this article are as follows:

� Demonstrate the need to improve the generalist’s performance ofclinical assessment in musculoskeletal medicine

� Suggest solutions within medical education and manpowerplanning in health care

� Focus on important areas of pMSK medicine to be learned by thegeneralist to help identify children with significant rheumaticdisease

� 2009 Elsevier Ltd. All rights reserved.

Fax: þ44 191 2225455.r), [email protected] (Y. Kimura).

td. All rights reserved.

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H. Foster, Y. Kimura / Best Practice & Research Clinical Rheumatology 23 (2009) 625–642626

The need to improve the generalist’s performance of paediatric musculoskeletal (pMSK)clinical assessment

MSK conditions are common in children and adolescents, affecting up to 30% of individuals [1,2].There is a broad spectrum of causes (Table 1), the majority of which are self-limiting, often traumarelated, and will not need referral to specialist care. However, these causes also include potentially life-threatening conditions, or may also be a feature of many chronic diseases, which often do need referral.The role of the generalist in the early recognition of new patients and the management of children withchronic rheumatic disease is integral to optimal care [3,4]. Children with MSK complaints often presentin various guises [5], and the generalist will have the responsibility to triage children. Appropriatetriage relies primarily on careful clinical assessment, with judicious use of investigations, knowledge ofcommon and medically significant conditions and awareness of local referral pathways. Unfortunately,evidence demonstrates that due to the large deficit in medical education and training that is prevalentcurrently, this process often breaks down; delay in access to specialist care is well recognised in manychildhood diseases, especially those with MSK presentations [6–10]. The reasons for this delay aremultifactorial, but likely include the poor pMSK clinical skills that are observed in physicians to whomchildren may present [11,12].

Care pathways for children with rheumatic disease vary within different health-care systems, andthe role of the generalist is changing in many of these. This is most apparent in the UK where there isa shift towards regional networks with paediatric rheumatology multidisciplinary teams (MDTs)working in specialist centres with outreach clinics linking with generalists using shared care clinicalguidelines and protocols (many of which are available at: http://www.bspar.org.uk/pages/

Table 1Common and significant causes of limping according to age.

Preschool� Infection (septic arthritis, osteomyelitis, Lyme arthritis)� Malignant disease (e.g. leukemia, neuroblastoma)� Mechanical (trauma and non-accidental injury)� Congenital/developmental problems (e.g. developmental dysplasia of the hip, talipes)� Neurological disease (e.g. cerebral palsy, neurological syndromes)� Juvenile Idiopathic Arthritis� Inflammatory muscle disease (e.g. inherited myopathies)� Metabolic disease (e.g. rickets)

Early School aged (4–10 years)� Mechanical (trauma, overuse injuries, sport injuries)� Reactive arthritis/Transient synovitis (‘‘irritable hip’’)� Infection (septic arthritis, osteomyelitis, Lyme arthritis)� Legg-Calves-Perthes disease� Juvenile Idiopathic Arthritis� Inflammatory muscle disease (e.g. juvenile dermatomyositis)� Tarsal coalition (painful non-mobile flat feet)� Complex diffuse/regional pain syndromes� Malignant disease (e.g. leukemia)� Metabolic disease (e.g. rickets)

Adolescence� Mechanical (trauma, overuse injuries, sport injuries)� Slipped Upper Femoral Epiphysis� Infection (septic arthritis, osteomyelitis, Lyme arthritis)� Juvenile Idiopathic Arthritis� Inflammatory muscle disease (e.g. juvenile dermatomyositis)� Lupus, inflammatory bowel disease and other autoimmune/inflammatory disorders causing arthritis� Complex diffuse/regional pain syndromes� Osteochonditis dissecans� Tarsal coalition� Complex diffuse/regional pain syndromes� Malignant disease (leukemia, lymphoma, primary bone tumor)� Metabolic disease (e.g. rickets)

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H. Foster, Y. Kimura / Best Practice & Research Clinical Rheumatology 23 (2009) 625–642 627

clinical_guidelines.asp) and aiming to deliver optimal clinical care locally. However, integral to thesuccess of these networks is having generalists who have adequate training and also have appropriatesupport from paediatric rheumatology MDTs. In many parts of the UK, however, there is inadequatesub-specialist service provision, the geographical coverage of clinical networks is variable and theresult is inequity of access to optimal care. In the US, there is currently no such planning of health-caredelivery and specialist care. When individual primary-care practitioners recognise significant diseasethat needs specialist care, the case is referred to specialists, who can be private practitioners or hospitaland/or university based. There are no state established regional centres for specialist care. Paediatricrheumatologists in the US are almost all based in large hospitals, often children’s hospitals, and accessis limited by multiple problems such as the paucity of specialists, insurance issues and geographicdistance. Similar inadequacy in pMSK specialist care is reported elsewhere and undoubtedly, for muchof the developing world, anecdotal evidence suggests that access to paediatric rheumatologists isseverely limited.

Generalist trainees and, unfortunately, even more experienced doctors are not confident in theirpMSK clinical skills [11,13–16] and this is likely to adversely affect access to care for children withrheumatic diseases. In the UK, the problem is compounded by recent changes in postgraduate training.All graduating medical students enter Foundation Programs (http://www.foundationprogramme.nhs;uk), which comprise structured modules over 2 years in general paediatrics, accident and emergencymedicine, primary care and surgery. Consequently, many new graduates will work in contact withchildren and likely to encounter children with MSK presentations, resulting in greater need foressential (core) pMSK clinical skills teaching during their undergraduate years.

Current pMSK teaching for undergraduates is suboptimal. Despite the mandatory inclusion ofclinical skills in UK undergraduate teaching (www.GMC.org.uk) [17], the focus to date has been onadult MSK clinical skills as part of core teaching [18], and evidence shows that it cannot be assumedthat MSK teaching in adults will translate into confident MSK assessment of children [11]. A recentsurvey of UK medical schools has shown that pMSK medicine is rarely included in general paediatricteaching or assessment. There are no specific pMSK learning outcomes, resulting in highly variablecontent between institutions, and teaching is often delivered by teachers who may have had littletraining in pMSK medicine themselves [19]. In North America, the situation is perhaps graver as thereare no published curricular guidelines that include adult or paediatric MSK learning objectives forundergraduate medical education or for postgraduate training programmes. A recent Canadian surveyreported an average less than 7 h devoted to teaching of MSK skills during the entire medical schoolcurriculum [20]. Not surprisingly, most American medical and surgical residents report being unpre-pared to conduct a MSK examination [21] and similar levels of low self-rated confidence and perfor-mance in pMSK clinical assessment have been reported in family medicine, paediatrics and accidentand emergency both in the UK and US [11–15,22].

Clearly, the generalist’s poor ability to recognise children with significant rheumatic disease isa major problem within medical education that is only beginning to be known. This comes at a timewhen early recognition and appropriate referral is more important than ever before, with the emer-gence of extremely efficacious treatments, and emerging evidence that early intervention improves theoutcome and prevents disability in these children.

Potential solutions within medical education and manpower planning

We believe that clinical skills in pMSK medicine must be integral to core paediatric clinical teachingat undergraduate level and all doctors, whatever their ultimate career path, should be trained in corepMSK clinical skills. A co-ordinated evidence-based strategy is necessary to define the content andstructure of core pMSK teaching, provide support to train the necessary personnel as teachers andevaluate the experience from both student and teacher perspectives. At the postgraduate level, thereneeds to be a programme of learning tailored to the needs of individual specialties and which builds oncore skills and knowledge acquired at medical school. However, there are currently multiple barriers topMSK clinical teaching that need to be addressed, including the ever-increasing pressures of a busycurriculum [23,24], lack of trained clinical teachers and the lack of standardised curricula.

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Table 2Musculoskeletal competences for the general paediatrician -sourced from the Royal College Paediatrics and Child Health(http://www.rcpch.ac.uk/Training/Competency-Frameworks).

B take an appropriate musculoskeletal history and examination (screening and approach to regional examination)B understand the association of musculoskeletal presentations and common chronic diseases (such as psoriasis,

inflammatory bowel disease)B know the differential diagnosis of musculoskeletal presentations including inflammatory, non-inflammatory and

idiopathic causesB recognize features in the clinical presentation or investigation findings which suggest serious pathology e.g.

inflammation, malignancy, infection and vasculitisB recognize features in the clinical presentation or investigation findings which suggest physical abuse, emotional

abuse and neglectB recognize when to request the opinion of a pediatric rheumatologist or orthopedic surgeonB understand the initial investigations to establish a diagnosisB understand the role of the multi-disciplinary team and other professionals involved in the care of children with

musculo-skeletal conditionsB understand the indication for and complications of immunosuppressive treatmentB understand the disease associations of rheumatological conditions, in particular juvenile arthritis and eye diseaseB be aware of congenital bone, inherited or metabolic conditions and their musculoskeletal presentationsB recognize the presentation of both chronic fatigue syndrome, generalized idiopathic pain syndromes and complex

regional pain syndromes

The patientpresents with

Knowledge and understanding Skills

Joint swelling � Know causes of joint swellingat single and multiple sites� Know when to refer for a

specialist opinion

� Take an appropriate musculoskeletal history� Be able to perform musculoskeletal

assessment including a screeningexamination and an approach tomore detailed examination� Be able to identify joint swelling and

abnormal range of joint movement� Be able to interpret the initial

investigations to establish a diagnosis

Musculoskeletal pain � Know the varied causes of musculoskeletalpain including referred pain and featuresthat suggest serious causes� Know the initial investigations to establish

a diagnosis� Know when to refer for a specialist opinion

� Take an appropriate musculoskeletalhistory� Perform a musculoskeletal examination

including a screening examinationand appropriate regional examination� Be able to interpret the initial

investigations to establish a diagnosis

Limp � Know the differential diagnosis of a limp atdifferent ages and clinical presentations� Know the initial investigations to establish

a diagnosis� Know when to refer for a specialist opinion

and when to involve pediatric rheumatologist

� Apply history and examination skillsin assessment� Be able to interpret the initial

investigations to establish a diagnosis

Limb pains Know the differential diagnosis of limb painsKnow the clinical features and be able to recognizebenign hypermobility and non-benignhypermobility (e.g. Marfan’s syndrome)

Apply history and examination skillsin assessmentBe able to assess joint laxityBe able to distinguish between inflammatoryand non-inflammatory conditions andrecognize features that suggestserious pathologyBe able to initiate and interpret investigationsthat may clarify the diagnosis

Back pain andneck painScoliosis

Know the causes of back/neck pain and be awareof features that may suggest seriousunderlying pathologyKnow the causes of scoliosisKnow the indications for further/urgentinvestigations and referral for specialist opinionKnow the common causes of torticollis

Apply history and examination skillsin assessmentBe able to recognize scoliosisBe able to initiate and interpret investigationsthat may clarify the diagnosis

H. Foster, Y. Kimura / Best Practice & Research Clinical Rheumatology 23 (2009) 625–642628

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Table 2 (continued)

The patientpresents with

Knowledge and understanding Skills

Leg alignment(normal variants)

Know the normal patterns of leg alignment andfoot posture (bow legs, knock knees, in-toeingand flat feet) at different agesBe aware of indications for investigation and whenspecialist opinion is requiredKnow the predisposing factors and presentationof rickets

Be able to assess clinically whether there islikely to be a serious underlying pathologyBe able to recognise the clinical presentationand radiological features of rickets

Multi-systemdisease

Know the clinical presentations and investigationsthat may suggest autoimmune diseaseKnow the initial investigations to establisha diagnosis

Be able to assess clinically whether there islikely to be a serious underlying pathologyand when specialist opinion is requiredBe able to initiate and interpret investigationsthat may clarify the diagnosis

H. Foster, Y. Kimura / Best Practice & Research Clinical Rheumatology 23 (2009) 625–642 629

Evidence-based pMSK learning outcomes

There is need to establish evidence-based consensus about pMSK learning outcomes for medicalstudents and also at postgraduate level. These will include clinical skills and knowledge relevant to thebroad spectrum of pMSK presentations (Table 1), red flags indicating serious disease (such as malig-nancy, infection, multisystem disease and non-accidental injury), whilst also addressing the approachto management and investigation. This includes knowledge regarding normal MSK developmentacross age groups, and common clinical scenarios in the community as well as acute presentationswithin hospital care. There is a great deal of clinical overlap among specialties in pMSK medicine(paediatric rheumatology, paediatric orthopaedics, sports medicine, rehabilitation medicine andphysical therapy), and so in developing consensus, it is vital that the process involves all stakeholderswith representation from all specialties as well as generalists to encompass different perspectives andreinforce relevance to clinical practice.

Inclusion of pMSK learning outcomes in undergraduate curricula

Standardised core curricula for medical schools do not exist in the UK or North America and,currently, institutions must deliver broad themes as outlined by the GMC (General Medical Council;www.GMC.org.uk) and the LCME (Liaison Committee on Medical Education: www.lcme.org), respec-tively. The attainment of clinical (including MSK) and practical skills is one of the integral principleswithin UK undergraduate teaching proposed by the GMC, although pMSK clinical skills are not specif-ically mentioned. The 2005 AAMC (Association of American Medical Colleges) White Paper outlinesMSK learning objectives (https://services.aamc.org/Publications/index.cfm?fuseaction¼Product.displayForm&prd_id¼204&prv_id¼245); however, these are recommendations rather than mandatory and thelisted MSK problems in children do not include arthritis. A core curriculum for the paediatric undergraduateclerkship in the US has been proposed and includes reference, albeit briefly, to pMSK clinical skills (Council onMedical Student Education in Pediatrics [http://www.comsep.org/Curriculum/CurriculumCompetencies/Skills.htm#Skills]). In Canada, a multidisciplinary approach has resulted in MSK themes for undergrad-uate medical education, which also includes pMSK topics, although they have yet to be implemented[25]. In the UK, under the auspices of the Royal College of Paediatrics and Child Health, a curriculum forchild health at undergraduate level is in development, and will include pMSK medicine and be evidencebased as much as possible. Implementing a syllabus that incorporates pMSK learning outcomes ischallenging, needs to address the ever-increasing pressures on the curriculum [24], interlink with otherparts of the course and be reflected in mandatory student assessment to drive learning [26].

Inclusion of pMSK learning outcomes in postgraduate training

Postgraduate learning builds on the core pMSK skills learned at medical school, although it must betailored to clinical practice and will vary between specialties; for example, the pMSK learning

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H. Foster, Y. Kimura / Best Practice & Research Clinical Rheumatology 23 (2009) 625–642630

outcomes for trainees in family medicine are likely to be different from those of trainees in emergencymedicine or orthopaedics. Again, consensus should be derived through involving all stakeholders, beevidence based where possible, be incorporated into mandatory assessments and involve trainees todiscern optimal teaching methodologies.

It is encouraging that currently there are numerous pMSK educational efforts in progress, which aimto address the needs of generalists. In the UK, the GP Learning Guide in rheumatology for primary care(www.arc.org.uk) is being revised to include evidence-based pMSK themes (Boyd, D personalcommunication), and the e-learning for Health (a National Health Service initiative to be launched late2009 http://www.rcgp.org.uk) includes pMSK modules. The Core Competency Frameworks for generalpediatricians (http://www.rcpch.ac.uk/Training) introduced in 2007, include pMSK clinical skills,knowledge and the importance of early referral to specialist care (Table 2); all paediatric trainees mustdemonstrate these competences and must also pass the mandatory written and clinical professionalexaminations, which have included pMSK themes since 2009. In North America, several individualinstitutional efforts are in progress and include educational resources related with pMSK themes andinclude case-based discussion with Web-streamed demonstration of MSK examination (www.rheum.ca) curricular guidelines for paediatric residents (Academic Pediatric Association Educational Guide-lines for Pediatric Residency, formerly the Ambulatory Pediatric Association – www.apa.org), and keyMSK themes (predominantly adult with some reference to pMSK) for postgraduate education [25].Clearly, there is more work to be done within emergency medicine, ophthalmology, orthopaedics andeven adult rheumatology, which currently have little mandatory training in paediatric rheumatology[27] although several collaborative efforts are underway. One notable multi-institutional, multidisci-plinary (rheumatology, orthopaedics and sports medicine) educational project involves a Web-basedmodular program called Pediatric Musculoskeletal Medicine, which aims to teach pMSK examinationand key knowledge through a multimedia format and is sponsored by the American Academy ofPediatrics (www.AAP.org) through a Web-based educational format called Pedialink� (to be launched2010).

Evidence-based pMSK educational tools to facilitate teaching and learning

The development of pMSK learning outcomes will drive the need for further educationalresources for medical students, postgraduate trainees and teachers. These resources must beevidence based where possible and be evaluated, although there are few validated educationalresources to date. The teaching of MSK clinical skills in adult patients has been facilitated by theintroduction of the GALS (Gait, Arms, Legs, Spine) MSK screening examination [28,29], which hasbeen validated [30,31], and REMS (Regional Examination of the Musculoskeletal System) [32],which are routinely taught at UK medical schools and facilitated by free educational resourcesbeing available (http://www.arc.org.uk/arthinfo/medpubs.asp). Emerging evidence shows thatGALS is learnt by students and is improving MSK performance amongst newly qualified doctors[33].

However, children are not little adults. This is exemplified by the testing of adult GALS in childrenwith rheumatic diseases, which demonstrated that GALS missed significant joint abnormalities inchildren [34]. Minor modifications to GALS based on simple manoeuvres used in clinical paediatricrheumatology practice resulted in the paediatric GALS (pGALS) MSK screening assessment (Table 3),which is the only MSK screening examination validated in school-aged children, with high sensi-tivity to detect significant abnormalities, quick to do and acceptable to both children and caregivers[34]. Furthermore, pGALS is practical to perform in acute paediatric settings and can be performedby non-experts in pMSK medicine (including medical students and family medicine doctors [Goffet al., in preparation]). pREMS (paediatric Regional Examination of the Musculoskeletal System) is anevidence-based approach to more detailed MSK examination, derived through involvement ofexperts in pMSK medicine and aimed at the postgraduate trainee [35]. Many MSK educationalresources are available through the Internet and although they present their own challenges [36],there is clearly enormous potential to raise the profile of pMSK medicine, engage with teachers andlearners across various health-care systems and promote pMSK clinical skills and knowledgeamongst generalists.

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Table 3pGALS - a musculoskeletal screening examination for school aged children - Table reproduced with permission from arthritisresearch campaign (arc) with further information and free educational resources available (http://www.arc.org.uk/arthinfo/emedia.asp).

The pGALS musculoskeletal screen– Table reproduced by kind permission of the Arthritis Research Campaign (www.arc.org.uk)Screening questions� Do you (or does your child) have any pain or stiffness in your (their) joints, muscles or back?� Do you (or does your child) have any difficulty getting yourself (him/herself) dressed without any help?� Do you (or does your child) have any problem going up and down stairs?

Screening maneuvers What is being assessed?

Observe the child standing (from front, back and sides) Posture, habitus, skin rashes, deformity – suchas leg length inequality, leg alignment (valgus,varus), scoliosis, joint swelling, muscle wasting,flat feet

Observe the child walking Ankles, subtalar, midtarsal, and small joints of feetand toes. Foot posture and presence of longitudinalarches of feet.

‘Hold your hands out straight in front of you’ Shoulders forward flexion, elbow extension, wristextension and extension of small joints of fingers

‘Turn your hands over and make a fist’ Supination of wrists and elbows, flexion of smalljoints of fingers

‘Pinch your index finger and thumb together Manual dexterity and coordination of small jointsindex finger and thumb

‘Touch the tips of your fingers’ Manual dexterity and coordination of small jointsof fingers and thumbs

Squeeze the metacarpophalangeal joints for tenderness Metacarpophalangeal jointsExtension of small joints of fingers, extensionof wrists, flexion of elbowsExtension of elbows and wrists, abductionof shoulders and extension of neck.

‘Put your hands behind your neck’ Abduction and external rotation of shoulders,flexion of elbows

Feel for effusion at the knee (patella tap, or cross fluctuation) Knee effusionActive movement of knees and feel for crepitus Knee flexion and extensionPassive movement (full flexion, internal rotation of hip Hip

Temporomandibular joints‘Try and touch your shoulder with your ear’ Cervical spine‘Bend forwards and touch your toes?’ Thoraco-lumbar spine

H. Foster, Y. Kimura / Best Practice & Research Clinical Rheumatology 23 (2009) 625–642 631

pMSK medicine should be taught by generalists and other health-care professionals

Clinical skills are integral to core paediatric clinical teaching at undergraduate level. We stronglybelieve that pMSK learning outcomes and especially core pMSK clinical skills need to be delivered bygeneralists to both reinforce their importance in general paediatric clinical practice and also toemphasise the differences from adults. This aim will be facilitated by the emergence of consensus-drivenpMSK learning outcomes and appropriate validated educational resources to aid the student and theteacher. Clearly there is need to teach the teachers and provide appropriate support to recruit generaliststo become core pMSK teachers. This is more likely achieved when generalists are working in clinicalnetworks delivering shared patient care and are supported by pMSK experts. There is also huge potentialfor other clinicians (e.g., physical therapists, sports medicine practitioners and nurse specialists), alongwith patients and parents, to take part in the delivery of pMSK teaching, expose students to the broadspectrum of pMSK medicine (not just acute scenarios in the hospital setting) and emphasise the impactof rheumatic diseases on the child and family.

Increasing manpower in pMSK medicine and education is critical

Having optimal numbers of pMSK sub-specialists (i.e., paediatric rheumatologists and paediatricorthopaedists) will enable delivery of optimal clinical care, further facilitate pMSK teaching and

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support the training of generalists working within clinical networks. However, currently, there isa significant manpower shortage in all the pMSK specialties worldwide and especially in paediatricrheumatology [3,4,37]. In the UK, the current provision of paediatric rheumatologists is suboptimaleven to recommendations from over a decade ago [38]. In the US, although the numbers of board-certified paediatric rheumatologists has increased in the US from 87 in 1990 to over 237 in 2006 [37],only one-third of US medical schools [39] and more than 40% of paediatric training programmes do nothave a paediatric rheumatologist on site [40], which is likely to limit pMSK teaching, trainee recruit-ment to the field and further perpetuate the manpower shortage.

Increasing the number of trained pMSK specialists, particularly paediatric rheumatologists, istherefore critical. The American College of Rheumatology is attempting to address this through severalprogrammes, such as the Paediatric Residents Programme to fund promising paediatric residents toattend its annual scientific meeting, the Pediatric Rheumatology Visiting Professorship programme, aswell as other preceptorship and research awards to students and residents (www.rheumatology.org).In the UK, there are currently 25 full-time paediatric rheumatologists, and clinical care is delivered inmany areas through clinical networks, although delay and inequity to high-quality clinical care isevident [6]. Moves to address this shortfall include promotion of the national Grid training scheme forpaediatric rheumatology (www.RCPCH/training.org.uk) through clinical networks, exposure to pMSKmedicine within the mandatory training for all paediatricians and promote the need for increasedfunding for clinical services though the recent Standards of Care (http://www.bspar.org.uk/downloads/clinical_guidelines/Standards_of_Care.pdf).

There is a potential to attract high-calibre recruits to paediatric rheumatology through the avail-ability of Student Selected Components, which are often a positive experience for students thatinfluence final career choice [41], and Student Elective and Exchange programmes. Undoubtedly, moreopportunities would arise with an increase in numbers of paediatric rheumatologists.

In summary, there are challenges that pose major implications for the organisation, funding anddelivery of pMSK education, but these are critical to overcome, and have a potential to improve pMSKclinical skills for all graduating doctors, many of whom will become generalists. These generalists will bethe ones who ultimately improve access to appropriate clinical care for children with rheumatic disease.

Research agenda

Improving pMSK Education Is Essential to Improving pMSK Clinical Skills

� pMSK learning outcomes need to be developed in a consensus driven, evidenced manner forwidespread (international) use in both undergraduate and postgraduate curricula� pMSK educational tools to facilitate teaching and learning that are evidence based need to be

developed and disseminated� Methods to rapidly increase manpower in pMSK medicine and education are urgently needed

to effectively implement pMSK educational initiatives

Clinical assessment approaches for the generalist

This section describes core pMSK clinical skills and knowledge that generalists should acquire toeffectively triage new patients presenting with MSK features and which need to be addressed in theirlearning.

Clinical skills

Taking an effective history and performing a competent physical examination are integral to pMSKclinical assessment. This necessitates knowledge of normal development, awareness of differences inclinical presentations at various ages and indicators that warrant investigation or urgent referral. TheMSK assessment of a child differs from that of an adult in several ways [42]; the history is often given bythe parent/caregiver, may be based on observations and interpretation of events made by others (such

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Table 4Distinguishing mechanical from inflammatory musculoskeletal presentations.

Mechanical Inflammatory

Pain Worse on weight bearing and afteractivity such as walking

Often eased by movement and may be seeminglyabsent in young children who and manifestas behavioural change or avoidance of activity

Joint swelling Usually mild and maybe transient Tends to be persistentMorning stiffness or gelling after rest Usually absent Often markedInstability (giving way) May be present Usually absentLocking May be present Usually absentLoss of full movement May be present Often present

H. Foster, Y. Kimura / Best Practice & Research Clinical Rheumatology 23 (2009) 625–642 633

as teachers or friends), and both parent and child may report rather vague and poorly localised reportssuch as ‘my leg hurts’, ‘my child is limping’ or ‘my child is not walking quite right’. The examination maybe challenging with a fractious child, and often necessitates an opportunistic approach. The parent orcaregiver will undoubtedly have anxieties and concerns, have an expectation of referral and investi-gations and these need to be addressed in the consultation.

History taking

A common diagnostic pitfall is to inappropriately attribute a child’s problem to trauma, given thatminor trauma is a common (almost daily) occurrence in all children. Open questions are helpful to teaseout features that may suggest a mechanical or inflammatory aetiology (Table 4) or red flags to warranturgent referral. The concept of referred pain should not be forgotten, for example, knee pain may bereferred from hip disease, or hip pain may be referred from the spine. Pre-verbal children of course cannotcomplain directly of pain, but even at an older age, the young child may deny having pain when askeddirectly, but this does not necessarily mean that pain is absent. The child in pain may simply present withchanges in behaviour or mood (e.g., irritability or poor sleeping), decreasing ability or interest in activitiespreviously enjoyed or functional limitations (e.g., drawing or painting, walking or running). Delay in majormotor milestones warrants a global neuro-developmental approach as well as MSK assessment. However,a history of regression of achieved milestones is often more suggestive of acquired MSK disease such as JIA;for example, the child who was previously happy to walk unaided but has recently been asking to becarried or refuses to go down the stairs unaided in the morning. A report of joint swelling is alwayssignificant, but is often not recognised by the parent (and even health-care professionals), especially if thechanges are symmetrical. Joint stiffness after inactivity followed by improvement with activity is commonin patients with inflammatory arthritis, but this particular complaint may be missing from the historywhen young children are involved. Rather than describing stiffness, parents may notice the child isreluctant to weight bear or limps in the mornings or ‘gels’ after periods of immobility (e.g., after long carrides or sitting in a classroom). Fever and other systemic symptoms and the presence of bone rather thanjoint pain may represent red flags that warrant urgent referral. Indolent presentations of chronic MSKdisease can impact on growth (either localised or generalised) and muscle wasting. It is important to assessheight and weight, review growth charts where available and share concerns with other health-careprofessionals (e.g., the school nurse and health visitor) and look for evidence of disproportionate growth(e.g., asymmetrical leg length) or muscle wasting.

Physical examination

A child who is in pain, frightened or very shy can result in physical examination being easier said thandone. Many children are put at ease by the availability of paper and crayons or blowing bubbles. Whilsttaking the history from the parent, it is valuable to observe the child at play, moving around the room andinteracting with their caregiver. Aside from a general assessment of the patient, a thorough generalphysical examination is essential. Clues may be missed if the examination only focusses on the area ofcomplaint; for example, the heart murmur discovered in a child who presents with knee pain and isultimately diagnosed with acute rheumatic fever. During the examination, it is also important to check fornon-verbal clues of joint discomfort, such as facial expression or withdrawal of limb, especially in children.

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The pGALS screening assessment takes an average of 2 min to perform (Table 3) and is aimed at theschool-aged child, but younger children will also often comply, especially if incorporated as play. ThepGALS screen includes three questions relating to pain and function, although it should be stressed thata negative response does not exclude significant MSK disease. At a minimum, pGALS should be done inall clinical scenarios where MSK disease is a concern, but is valuable as a core skill in the assessment ofall children, and ideally should be done as part of a physical examination in all children, much like theheart and lung examinations. In addition, because the history alone can underestimate the degree ofjoint involvement [43], it is essential to perform all parts of the pGALS screen; it is not unusual fora child referred with a swollen knee to have multiple abnormal joints which were not detected by otherassessors because the focus was only on the knee. For the specialist trainee in pMSK medicine, there aremore detailed descriptions of the clinical examination [44], but the generalist observer is suggested tofollow the screening examination with a more detailed examination of the relevant area, based on the‘look, feel, move’ principle as in the adult Regional Examination of the Musculoskeletal system (calledREMS [32] http://www.arc.org.uk/arthinfo/medpubs/6321/6321.asp), although an evidence-basedapproach to a children’s regional examination to be called pREMS, (paediatric Regional Examination ofthe Musculoskeletal System) has been developed [35].

Lastly, it is important for the generalist to know normal variants in gait, leg alignment and normalmotor milestones (Table 5) as these are a common cause of parental concern, especially in the pre-school child, and often can be managed simply with explanation and reassurance.

Common clinical scenarios

The limping childThe limping child is displaying a classical sign of MSK impairment or pain. Limp is usually defined as

an abnormality in gait, usually caused by pain, deformity or weakness and various types of limp aredescribed [45]. Limping has a variety of causes (Table 1) which vary from life threatening to benign andusually can be differentiated by careful clinical assessment. The initial evaluation should be to ascertainthe general appearance of the child and whether s/he appears well, is playing and is able to ambulate;

Table 5Common normal variants in gait and leg alignment. Table reproduced with permission from Arthritis Research Campaign - ‘‘pGALS,a musculoskeletal screening examination for school aged children’’ - (http://www.arc.org.uk/arthinfo/documents/6535.pdf).

Normal variants in gait patterns and leg alignmentHabitual toe walking is common in young children up to 3 years.In toeing can be due to:� persistent femoral ante version (characterized by child walking with patellae and feet pointing inwards and is common

between ages of 3–8 years)� internal tibial torsion (characterized by child walking with patella facing forward and toes pointing inwards and is

common from onset of walking to 3 years)� metatarsus adductus (characterized by a flexible ‘C shaped’ lateral border of the foot and most resolve by 6 years).

Bow legs (genu varus) are common from birth to early toddler, often with out-toeing (maximal at approximately 1 year of age),and most resolve by 18 months.

Knock knees (genu valgus) are common and are often associated with in-toeing (maximal at approximately 4 years of age)and most resolve by age of 7 years.

Flat feet – most children have a flexible foot with normal arch on tiptoeing and resolve by 7 years.

Crooked toes – most resolve with weight bearing.

Practical Tip – normal variants: indications for referral� Persistent changes (beyond the expected age ranges)� Progressive or asymmetrical changes� Short stature (especially short limbs) or dysmorphic features� Painful changes with functional limitation� Flat feet if fixed (i.e. non mobile) or painful� High fixed foot arches� Regression or delayed motor milestones� Abnormal joint examination elsewhere� Suggestion of neurological disease

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H. Foster, Y. Kimura / Best Practice & Research Clinical Rheumatology 23 (2009) 625–642 635

this is easily done by observing the child whilst the history is being taken. Suspicion of a limp may arisefrom the pGALS assessment or from the history and should be followed up with the child being askedto walk normally up and down a long hallway; it is important to get the child to do this at least a fewtimes so that any initial hesitance or embarrassment dissipates. The non-weight bearing or the ill-looking child in severe pain warrants careful assessment to exclude life- or limb-threatening disorderssuch as a bacterial infection (osteomyelitis or septic arthritis) and malignancies.

Warning signs. Red flags that increase the likelihood of infection include fever with involvement of onlyone joint or bone and severe pain in one location causing inability to weight bear or restriction orreluctant to move a joint or limb. Severe bone pain can also occur in malignancies such as leukaemia andmetastatic neuroblastoma, although very often these involve multiple sites. Traumatic injury is anothermajor cause of limp, but as mentioned earlier, a common mistake is to ascribe the limp inappropriately toa minor injury. Generally, limping due to trauma is almost always secondary to a major identifiable event,which is immediately followed by swelling, bruising or severe pain in the affected extremity.

Rheumatology versus orthopaedics. Obvious traumatic injuries are of course best referred to orthopaedists,but patients with insidious onset of pain are more likely to have a rheumatologic disorder and should bereferred to a rheumatologist for evaluation. The exception is when there are symptoms that make the painlikely to be due to a mechanical or overuse disorder, such as pain mainly related to or worsened by physicalactivity, locking or giving way of a joint, which should be referred to orthopaedics. Other considerationsinclude any relationship to preceding illnesses (as in reactive arthritis or acute rheumatic fever) or physicalactivity (pain worsened by inactivity and improved by moderate activity is common in inflammatoryarthritis, whereas pain occurring only after activity suggests mechanical disorders [Table 4]).

A child who has indications of a systemic disorder with multi-organ system involvement is morelikely to have a rheumatologic cause of MSK pain or limp. Determination of whether there are otherclues that may be related to the illness will help in putting together a pattern of symptoms that shouldlead to the correct diagnosis, such as rash or other skin findings (e.g., lupus, Henoch Schonlein Purpura,dermatomyositis and vasculitis), proximal muscular weakness (dermatomyositis and other myopathy),dysmorphic features (skeletal dysplasias and Marfan syndrome), abdominal pain and diarrhoea(inflammatory bowel disease, coeliac disease) and a combination of hair loss, pancytopenia andproteinuria (lupus) are just a few examples.

Practice points

Clues to help distinguishing the cause of MSK Symptoms being Rheumatological orOrthopaedic

Cause likely to be Orthopaedic:

� Immediate pain and swelling following traumatic injury� Mechanical symptoms (pain clearly related to increased physical activity, locking or giving way

of joints)� Fever and severe pain and/or swelling of one joint or bone (likely bacterial infection)

Cause likely to be Rheumatological:

� Symptoms indicative of inflammatory arthritis (Pain and/or stiffness is worse after periods ofinactivity or in the morning, and gets better with moderate activity and regression of achievedmotor milestones.)� Joint swelling not clearly related to traumatic injury� Systemic symptoms (fever, weight loss, rash and other organ system involvement)

Age of the patient. Aside from these major considerations, the age of the patient is an important factor.Although many conditions can present with limping at any age, some are more common in certain agegroups (Table 1). For example, in terms of disorders causing hip pain and limping, transient or toxic

Page 12: Ensuring that all paediatricians and rheumatologists recognise significant rheumatic diseases

Reproduced and adapted with permission from Oxford University Press Y Kimura. “Common Presenting Problems,” in IS Szer, Y Kimura, PN Malleson and TR Southwood, eds., Arthritis in Children and Adolescents. Oxford, UK: Oxford University Press 2006.

*IMPORTANT NOTES: Children with persistent musculoskeletal pain should have the following work-up considered:

Complete/full blood count with differential Acute phase reactants (ESR, C-reactive protein) Routine serum chemistries (including creatinine, liver and muscle enzymes) Urinalysis Imaging studies (plain X-rays of involved area, other studies as indicated in the algorithm(s)) Testing for mycobacterial infection

**CAVEATS: Minor trauma and infections are common in children and may not necessarily be related to the diagnosis Remember infection, malignancy and non-accidental injury (child abuse) at each stage

AFEBRILE CHILD WITH MUSCULOSKELETAL PAIN/DYSFUNCTION *

Does the child appear ill, or refuse to weight bear?

Is there a rash?

Is there weakness, loss of sensation or other neurological symptom?

Has there been a history of significant trauma or evidence of trauma on exam?**

Was there an antecedent infection in the month before pain began?**

Is pain limited to the hip or anterior thigh / knee?

Consider dermatologic manifestations of rheumatic diseases (i.e., HSP, lupus,

dermatomyositis, vasculitis)

Consider inflammatory myositis if proximal weakness, neurologic problem or neurogenic

pain Discuss appropriate imaging studies with

radiologist& neurologist

Consider isolated hip diseases (Legg Perthes, Transient synovitis, Slipped capital femoral

epiphysis depending on the age)

Consider reactive arthritis and other infection related arthritis (remember acute rheumatic

fever), and sexually acquired infection in older pt

Consider traumatic injury (remember non-accidental injury) and referral to an orthopaedic

specialist

Consider bacterial MSK infection or malignancy Go to Figure 2 Algorithm: The Child with Fever

and Musculoskeletal Pain or Limping

no

yes

no

no

no

no

no

yes

yes

yes

yes

yes

Fig. 1. Algorithm: The Child with Musculoskeletal Pain or Limping (without Fever).

H. Foster, Y. Kimura / Best Practice & Research Clinical Rheumatology 23 (2009) 625–642636

Page 13: Ensuring that all paediatricians and rheumatologists recognise significant rheumatic diseases

Consider causes of chronic arthritis (Juvenile Idiopathic Arthritis and other

inflammatory/rheumatic conditions such as lupus, dermatomyositis, vasculitis, inflammatory bowel

disease, etc.)

Is there joint swelling, tenderness, loss of motion or pain on motion associated

with morning stiffness? yes

Is there short or tall stature, excessive hypermobility, dysmorphic features or family history of similar symptoms?

Consider Munchausen or Munchausen by

proxy Suggestive symptoms:

Bizarre physical signs Disparity between reported and observed signs

Signs in presence of carer

Consider Somatization Suggestive symptoms:

Pain in excess of physical signs

Parent – child disparity

School avoidance Stress

Consider localized pain syndromes such as

Complex Regional Pain Syndrome I if pain is

localized to one extremity Suggestive symptoms:

Hyperaesthesia/allodynia Hypervigilance Bizarre posture Normal blood count/ESR NB may have swelling (usually diffuse)

Consider Fibromyalgia if pain is diffuse

Suggestive symptoms: Fatigue Sleep disturbance Symmetrical tender points

Pain in excess of physical signs

Normal blood count/ESR

Consider inherited causes of MSK pain (skeletal dysplasias, connective tissue diseases such as Marfans

or Ehlers Danlos)

no

yes

Are there symptoms compatible with a metabolic or endocrine

disorder?

Consider MSK symptoms associated with rickets, endocrinopathies, i.e.,hypo- or hy perthyroidism,

diabetes, Cushing or Addison disease yes

no

no

Is the pain only in the evening or night and not associated with any

MSK dysfunction?

Consider Growing Pains (see Table7) yes

Is the pain mainly related to or worsened with physical activity?

Consider mechanical joint/bone problems (see Table 4) yes

no

no

Fig. 1. (continued)

H. Foster, Y. Kimura / Best Practice & Research Clinical Rheumatology 23 (2009) 625–642 637

synovitis is more common in the pre-school age group, but in the school-aged child, Legg–Calve–Perthes disease is more common, while in the adolescent, slipped capital/upper femoral epiphysis(SCFE or SUFE) is more likely. Another example is that while leukaemia can present with musculo-skeletal pain at any age, neuroblastoma is more common in the toddler and lymphoma and primarybone tumours are more common in the adolescent.

An algorithm outlining the major considerations to think through is presented in Fig. 1: Algorithm AThe child with musculoskeletal pain or limping (without fever).

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Table 6The ‘‘rules’’ of growing pains. Table reproduced by kind permission of the Arthritis Research Campaign (www.arc.org.uk).

Pains never present at the start of the day after waking� Child doesn’t limp� Physical activities not limited by symptoms� Pains symmetrical in lower limbs and not limited to joints� Physical examination normal (with the exception of joint hypermobility)� Systemically well� Gross motor milestones normal� Age range 3–12 yrs

H. Foster, Y. Kimura / Best Practice & Research Clinical Rheumatology 23 (2009) 625–642638

Special clinical scenarios. Hypermobility is a common cause of MSK discomfort in children. It may begeneralised or limited to peripheral joints such as hands and feet. Generally speaking, younger femalechildren and those of non-Caucasian origin are more flexible than adults. Hypermobility is suggested bysymmetrical hyperextension at the fingers, elbows, knees and flat pronated feet, with normal arches ontiptoe. Childrenwith benign hypermobility syndrome may present with mechanical aches and pains whichoccurafter increased activity (and are more common in childrenwith growing pains as outlined below). It isimportant, however, to consider and exclude inherited collagen disorders associated with hypermobilitysuch as Marfanoid syndromes, which although rare, associate with retinal and cardiac complications.

Growing pains is a common diagnosis given to children with non-specific MSK pains. Despite therebeing no clear association with growth, the term has become embedded in medical literature andreflects our poor understanding of this condition. Far too often, children with MSK pain are givena vague diagnosis of growing pains rather than being given the benefit of further investigation bygeneralists who are not confident in MSK assessment. However, growing pains is a recognised clinicalsyndrome with clear parameters for making the diagnosis (Table 6) and guiding management (http://www.arc.org.uk/arthinfo/medpubs/6541/6541.asp). They are a common clinical entity with equalgender preponderance, characterised by aches and pains poorly localised in the calves, shins, feet andankles and not always focussed on a joint [46]. Typically, parents report no obvious swelling orbruising, the pains are relieved with massage or simple analgesics and are often predictable to occurlater in the day, evenings and after periods of activity. If these rules of growing pains do not fit a givenclinical situation, then it is important to consider other diagnostic possibilities. In the majority of cases,however, if a child has a typical presentation consistent with the rules, no investigations are necessary.

Fever and joint pain or limpingWhen fever accompanies limping or joint pain, especially if only a single joint or bone is involved,

bacterial infection becomes a much more likely possibility. Again, severe joint pain with swelling, warmthor redness, inability to bear weight or point bone tenderness are red flags that dictate immediate investi-gation with arthrocentesis and or bone aspiration, as well as blood counts, acute-phase reactants and bloodcultures. Imaging with radiographs should be done as a baseline and to rule out unsuspected bone lesions ortrauma (in most cases of acute septic arthritis and osteomyelitis, radiographs will initially be normal). Ifthere is more than one joint involved, the likelihood of an inflammatory disorder increases, but malignancyshould also be considered. Again, inability to weight bear, ill appearance, as well as other signs such aslymphadenopathy, weight loss and splenomegaly are suggestive. Laboratory tests suggestive of leukaemiainclude pancytopenia, a low normal platelet count, despite an elevated erythrocyte sedimentation rate, anda highly elevated lactate dehydrogenase, even in the absence of lymphoblasts on the blood film [47–49].

Practice points

Warning Signs (red flags) Indicative of Urgent Referral and Investigation

� Monoarticular involvement� Refusal to bear weight� Fever or other systemic symptoms� Ill appearance

Page 15: Ensuring that all paediatricians and rheumatologists recognise significant rheumatic diseases

Reproduced and adapted with permission from Oxford University Press Y Kimura. “Common Presenting Problems,” in IS Szer, Y Kimura, PN Malleson and TR Southwood, eds., Arthritis in Children and Adolescents. Oxford, UK: Oxford University Press 2006.

*IMPORTANT NOTES: Children with significant continued fever and musculoskeletal pain should have the following during the work-up:

Complete blood count with differential Acute phase reactants (ESR, C-reactive protein) Routine serum chemistries (including creatinine, muscle and liver enzymes) Urinalysis ASO titre Cultures as appropriate (blood, urine, throat); if considering endocarditis, obtain at least 2 blood culturesfrom separate sites (remember that endocarditis can present with polyarthritis and fever) Testing for mycobacterial infection Imaging studies of involved area (plain X-rays, other tests as indicated in algorithm) Immunoglobulins and other immunodeficiency studies as appropriate

One joint

Strongly consider arthrocentesis:

Purulent joint fluid, positive Gram stain and/or

culture?

INFECTION Treat for septic arthritis

or aspirate bone and treat for osteomyelitis

Possible osteomyelitis, discitis or malignancy?

Discuss further imaging studies with radiologist

Treat according to diagnosis

More than one joint: Likely an inflammatory disorder, but need

to go through algorithm

MALIGNANCY Bone marrow

aspiration/biopsy or other diagnostic testing

as appropriate

A CHILD WITH FEVER AND MUSCULOSKELETAL PAIN OR LIMPING *

Yes

No

Appears ill, refuses to bear weight or has bony point tenderness?

If none of the above but fever & pain continue: Go to “Diagnosis still unknown” below

Yes

No

Yes, could be osteomyelitis Yes, could be malignancy

None

Number of involved joints

Fig. 2. Algorithm: The Child with Fever and Musculoskeletal Pain.

H. Foster, Y. Kimura / Best Practice & Research Clinical Rheumatology 23 (2009) 625–642 639

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CONSIDER PSYCHOGENIC CAUSES

OR IDIOPATHIC Factitious fever and/or

other psychogenic illness; Continue to monitor and

consider other conditions if symptoms continue

Complete FUO Work-up: Repeat blood and urine cultures;

Chest X-ray, CT abdomen/pelvis, gallium or bone scan Consider bone marrow aspiration (for morphology and cultures)

If indicated, lymph node biopsy and/or GI work-up for IBD

CONSIDER EXTRA-ARTICULAR INFECTION

such as endocarditis, pyomyositis, perinephric,

psoas, renal or other intra-abdominal abscess, etc.

LOOK FOR ADDITIONAL CLUES (Look for symptom and fever patterns, rash, other organ system involvement)

Remember Kawasaki Disease, Autoinflammatory Disorders (Periodic

Fever Syndromes) If diagnosis still unknown & symptoms

still present, continue algorithm

Consider SYSTEMIC JIA* and referral to a

pediatric rheumatologist

Consider OTHER CHRONIC RHEUMATIC OR INFLAMMATORY CONDITIONS (i.e,. lupus, dermatomyositis,

IBD, vasculitis, sarcoidosis, etc.) and referral to a pediatric rheumatologist

Child continues to have musculoskeletal pain and fever Check or repeat CBC, ESR, CRP, chemistry panel, urinalysis

Blood, urine cultures (Obtain Ferritin and D-dimer if Systemic JIA strongly suspected)

DIAGNOSIS STILL UNKNOWN: Consider infection syndromes associated with infection. If not

consistent, go to next step

Negative cultures but elevated APR

Negative cultures and normal acute phase

reactants (APR)

Positive culture(s)

Patient has arthritis, typical systemic rash, and quotidian fever pattern

Patient has arthritis but does not have typical systemic rash and quotidian fever pattern

FUO work-up is negative and diagnosis is still unknown

Fig. 2. (continued)

H. Foster, Y. Kimura / Best Practice & Research Clinical Rheumatology 23 (2009) 625–642640

Symptom and fever patterns. Further, it is important to look for symptom patterns, and to do a completephysical examination to localise the pain and assess for other signs and symptoms that may be clues tothe diagnosis. In addition, an assessment of the fever pattern is also important. Although Kawasakidisease and bacterial infections tend to be associated with persistently elevated high-grade fever, otherillnesses in which fever is not necessarily a key feature (e.g., lupus, other types of vasculitis andinflammatory bowel disease) may not have specific fever patterns. Auto-inflammatory disorders, in

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contrast, usually have highly specific fever patterns. For example, patients with Familial MediterraneanFever tend to have brief episodes (2–3 days) of fever often accompanied by abdominal pain, joint painand or erysipeloid rash, often occurring every few weeks, with entirely normal periods in betweenfever episodes. Other types of periodic fever syndromes have distinctive fever patterns, and the key todiagnosis is to recognise the pattern and the periodicity to the fevers. Likewise, patients with systemicJIA have a quotidian fever pattern (one spike of high fever a day often in the late afternoon or evening),usually accompanied by the appearance of the typical rash, with normal or subnormal temperatures inbetween the spikes. An algorithm outlining the approach to the assessment of a child with limping andfever is given in Fig. 2: Algorithm B (The Child with Fever and Musculoskeletal Pain).

In summary, despite the fact that MSK conditions are common in childhood, there is currentlya major knowledge deficit among generalists in the assessment and management of children andadolescents with MSK disorders. This leads to misdiagnosis, delayed diagnosis and initiation oftreatment, with potential for poorer outcome. This deficit in medical education needs to be addressed,beginning with the addition of specific MSK curricula (adult and paediatric) in undergraduate medicaleducation, increased and formalised exposure to pMSK medicine in training programmes andincreasing the number of pMSK medicine-trained specialists. Teaching should include specific physicalexamination skills in pMSK medicine starting with pGALS as well as more detailed pMSK examinationskills. All physicians caring for children should be taught how to discriminate between various causesof MSK abnormalities in children, to distinguish pathology from normal developmental changes and toknow what signs and symptoms should warrant referral to rheumatologists.

References

[1] Yeo M, Sawyer S. Chronic illness and disability. BMJ 2005 Mar 26;330(7493):721–3.[2] Goodman JE, McGrath PJ. The epidemiology of pain in children and adolescents – a review. Pain 1991 Sep;46(3):

247–64.*[3] Mayer ML, Sandborg CI, Mellins ED. Role of pediatric and internist rheumatologists in treating children with rheumatic

diseases. Pediatrics 2004 Mar;113(3 Pt 1):e173–81.*[4] Mayer ML, Mellins ED, Sandborg CI. Access to pediatric rheumatology care in the United States. Arthritis Rheum 2003 Dec

15;49(6):759–65.[5] McGhee JL, Burks FN, Sheckels JL, Jarvis JN. Identifying children with chronic arthritis based on chief complaints: absence

of predictive value for musculoskeletal pain as an indicator of rheumatic disease in children. Pediatrics August 1, 2002;110(2):354–9. doi:10.1542/peds.110.2.354.

*[6] Foster HE, Eltringham MS, Kay LJ, et al. Delay in access to appropriate care for children presenting with musculoskeletalsymptoms and ultimately diagnosed with juvenile idiopathic arthritis. Arthritis Rheum 2007 Aug 15;57(6):921–7.

[7] Manners PJ. Delay in diagnosing juvenile arthritis. Med J Aust 1999 Oct 4;171(7):367–9.[8] Dang-Tan T, Franco EL. Diagnosis delays in childhood cancer: a review. Cancer 2007 Aug 15;110(4):703–13.[9] Mohamed K, Appleton R, Nicolaides P. Delayed diagnosis of Duchenne muscular dystrophy. Europ J Paediatr Neurol 2000;

4(5):219–23.*[10] Shiff NJ, Abdwani R, Cabral DA, et al. Access to pediatric rheumatology subspecialty care in British Columbia,

Canada. J Rheumatol 2009 Feb;36(2):410–5.[11] Jandial S, Myers A, Wise E, Foster HE. Doctors likely to encounter children with musculoskeletal complaints have low

confidence in their clinical skills. J Pediatr 2009 Feb;154(2):267–71.[12] Myers A, McDonagh JE, Gupta K, et al. More ‘cries from the joints’: assessment of the musculoskeletal system is poorly

documented in routine paediatric clerking. Rheumatology August 1, 2004;43(8):1045–9.[13] Bonds DE, Mychalecky JC, Watkins R, et al. Ambulatory care skills: do residents feel prepared? Med Educ Online 2002;

7(7).[14] Glazier RH, Dalby DM, Badley EM, et al. Determinants of physician confidence in the primary care management of

musculoskeletal disorders. J Rheumatol 1996 Feb;23(2):351–6.[15] Hergenroeder AC, Chorley JN, Laufman L, Fetterhoff AC. Pediatric residents’ performance of ankle and knee examinations

after an educational intervention. Pediatrics 2001 Apr;107(4):E52.[16] Lanyon P, Pope D, Croft P. Rheumatology education and management skills in general practice: a national study of

trainees. Ann Rheum Dis 1995 Sep;54(9):735–9.[17] GMC. Good medical practice. 2001; London.

*[18] Kay LJ, Deighton CM, Walker DJ, Hay EM. Undergraduate rheumatology teaching in the UK: a survey of current practiceand changes since 1990. Rheumatology July 1, 2000;39(7):800–3.

*[19] Jandial S, Rapley T, Foster H. Current teaching of paediatric musculoskeletal medicine within UK medical schools–a needfor change. Rheumatology (Oxford) 2009 May;48(5):587–90.

[20] Oswald AE, Bell MJ, Snell L, Wiseman J. The current state of musculoskeletal clinical skills teaching for preclerkshipmedical students. J Rheumatol 2008 Dec;35(12):2419–26.

[21] Clawson DK, Jackson DW, Ostergaard DJ. It’s past time to reform the musculoskeletal curriculum. Acad Med 2001 Jul;76(7):709–10.

[22] Dubey SG. Musculoskeletal assessment of general medical inpatients. Rheumatology (Oxford) 2004 Apr;43(4):536–7.author reply 7.

Page 18: Ensuring that all paediatricians and rheumatologists recognise significant rheumatic diseases

H. Foster, Y. Kimura / Best Practice & Research Clinical Rheumatology 23 (2009) 625–642642

[23] Craze J, Hope T. Teaching medical students to examine children. Arch Dis Child 2006 Dec;91(12):966–8.[24] Gillam S, Bagade A. Undergraduate public health education in UK medical schools - struggling to deliver. Med Educ 2006

May;40(5):430–6.[25] Wadey VM, Tang ET, Abelseth G, et al. Canadian multidisciplinary core curriculum for musculoskeletal health. J Rheu-

matol 2007 Mar;34(3):567–80.[26] Newble DI, Jaeger K. The effect of assessments and examinations on the learning of medical students. Med Educ 1983

May;17(3):165–71.[27] Giannini EH, Ruperto N, Athreya B, et al. Specialty training and distribution of work effort among US American College of

Rheumatology members caring for children with rheumatic disease. Arthritis Rheum 1997 Dec;40(12):2273–4.[28] Doherty M, Dacre J, Dieppe P, Snaith M. The ‘GALS’ locomotor screen. Ann Rheum Dis 1992 Oct;51(10):1165–9.[29] Jones A, Ledingham J, Regan M, Doherty M. A proposed minimal rheumatological screening history and examination. The

joint answers back. J R Coll Physicians Lond 1991 Apr;25(2):111–5.[30] Beattie KA, Bobba R, Bayoumi I, et al. Validation of the GALS musculoskeletal screening exam for use in primary care:

a pilot study. BMC Musculoskelet Disord 2008;9:115.[31] Lillicrap MS, Byrne E, Speed CA. Musculoskeletal assessment of general medical in-patients–joints still crying out for

attention. Rheumatology August 1, 2003;42(8):951–4.[32] Coady D, Walker D, Kay L. Regional Examination of the Musculoskeletal System (REMS): a core set of clinical skills for

medical students. Rheumatology May 1, 2004;43(5):633–9.[33] Fox RA, Dacre JE, Clark CL, Scotland AD. Impact on medical students of incorporating GALS screen teaching into the

medical school curriculum. Ann Rheum Dis 2000 Sep;59(9):668–71.*[34] Foster HE, Kay LJ, Friswell M, et al. Musculoskeletal screening examination (pGALS) for school-age children based on the

adult GALS screen. Arthritis Rheum 2006 Oct 15;55(5):709–16.*[35] Rapley TJ, Heaven B, May CR, et al. An evidence and practice based regional musculoskeletal examination for school aged

children – pREMS’. Rheumatology (Oxford) 2009;48(1. i22).[36] Cividino A. The challenges of developing online learning. J Rheumatol 2009 Mar;36(3):470–1.[37] Althouse LA, Stockman 3rd JA. Pediatric workforce: a look at pediatric rheumatology data from the American Board of

Pediatrics. J Pediatr 2006 Dec;149(6):869–70.[38] Recommendations for paediatric rheumatology service in the UK: British Paediatric Association; 1994.[39] Cassidy JT, Athreya B. Pediatric rheumatology: status of the subspecialty in United States medical schools. Arthritis

Rheum 1997 Jun;40(6):1182.*[40] Mayer ML, Brogan L, Sandborg CI. Availability of pediatric rheumatology training in United States pediatric residencies.

Arthritis Rheum 2006 Dec 15;55(6):836–42.[41] Kolasinski SL, Bass AR, Kane-Wanger GF, et al. Subspecialty choice: why did you become a rheumatologist? Arthritis Care

Res 2007;57(8):1546–51.[42] Foster HE, Cabral DA. Is musculoskeletal history and examination so different in paediatrics? Best Pract Res Clin Rheu-

matol 2006 Apr;20(2):241–62.[43] Goff I, Rowan AR, Bateman B, et al. Sensitivity of musculoskeletal history taking in children is poor; the need to examine

all joints. Rheumatology (Oxford) 2009;48(Supp 1. i92).*[44] Szer I. Clinical skills in the evaluation of arthritis. In: Szer I, Kimura Y, Malleson PN, Southwood TR, editors. Arthritis in

children and adolescents. Oxford, UK: Oxford University Press; 2006.[45] Beresford MW, Cleary AG. Evaluation of a limping child. Curr Paediatr 2005;15:15–22.[46] Peterson HA. Leg aches. Pediatr Clin North Am 1977 Nov;24(4):731–6.[47] Cabral D, Tucker L. Malignancies in children who initially present with rheumatic complaints. J Pediatr 1999;134:53–7.[48] Jones OY, Spencer CH, Bowyer SL, et al. A multicenter case-control study on predictive factors distinguishing childhood

leukemia from juvenile rheumatoid arthritis. Pediatrics 2006 May;117(5):e840–4.[49] Wallendal M, Stork L, Hollister JR. The discriminating value of serum lactate dehydrogenase levels in children with

malignant neoplasms presenting as joint pain. Arch Pediatr Adolesc Med 1996 Jan;150(1):70–3.