STAFF CONFERENCE

SEPTEMBER 19, 2008MARIA KARLA C. SAN PEDRO, MD

Objectives• Determine the approach to a child with joint swelling

• Be familiar with Relapsing Polychondritis as a differential diagnosis for joint swelling

• Differentiate Relapsing Polychondritis from other arthritides with systemic manifestations

• Know the diagnosis and management of Relapsing Polychondritis

Our Patient D.C., 6 /M From Meycauayan, Bulacan First admission on July 23, 2008 Chief complaint: right ear swelling

History of Present Illness 9 months PTA

Right ankle pain after tripping on the street, grade 4/10, associated with swelling, warmth, low grade fever; difficulty in ambulation

Given Paracetamol but with no reliefBrought to Philippine Orthopedic Center;

casting done; no relief

History of Present Illness 8 months PTA

Increase in severity of pain of right ankle, grade 7/10 accompanied with swelling of the nose, tender, warm

Brought to a local health center and given Cefalexin x 5 days with partial relief

7 months PTAFell from his bike; swelling of left ankle with

superficial abrasions

History of Present Illness 6 months 3 weeks PTA

Left knee swelling accompanied by difficulty in ambulation

Brought to a private doctor and given Cloxacillin x 7 days with temporary relief

History of Present Illness 6 months PTA

Persistence of difficulty in ambulation Brought to Philippine Orthopedic CenterA> Septic Arthritis of the left kneeUnderwent Incision and Drainage and arthrotomy

of the left knee and left ankle; purulent material on drainage of fluid

Given Oxacillin; Biopsy: granulomatous inflammation; started on Anti Koch’s; discharged apparently improved

History of Present Illness 3 months PTA

Brought to Philippine Orthopedic Center for swelling of both wrists and elbows accompanied by undocumented fever

Residual purulent material on the left knee and ankle; advised admission but opted to go home

History of Present Illness 1 month PTA

Increase in severity of symptomsBrought to Mary Johnston HospitalA> Juvenile Idiopathic ArthitisGiven Methotrexate

History of Present Illness 6 days PTA

Enlargement of the right ear with redness and itchiness associated with swelling and tenderness of both wrists, both elbows, left knee, and left ankle

Brought to a private doctor and referred to PGH Rheumatology

Review of Systems(+) Oral ulcers x 6

days(-) Genital ulcers(-) Dyspnea (-) Dysphagia(-) Epistaxis (-) Colds

(-) Blurring of vision(-) Vomiting(-) Raynaud’s

phenomenon(-) Weight loss(-) Oliguria,

Hematuria

Past Medical History Dengue Hemorrhagic Fever – 3 y/o Mumps – 5 y/o No previous Blood transfusions No previous operations

Family Medical History(+) Hypertension – paternal aunt(-) DM, PTB, BA, Kidney disease, Liver disease

Birth and Maternal History FT via SVD to a 29 year old G3P2 (2002) mother

at home c/o traditional birth attendant; mother with regular PNCU c/o LHC

Mother had frequent cough and colds during pregnancy; treated with Paracetamol with relief; no intake of teratogenic drugs, no exposure to radiation

At birth, patient had good cry and activity; no fetomaternal complications

Nutritional History Breastfed until 2 months old Shifted to formula feeding with Bonna at

2 months to 1 year old Complementary feeding at 6 months old No food preferences

Immunization History (+) BCG, OPV3, DPT3, Hepa B3, measles

Developmental History At par with age

Personal and Social History Youngest of 3 children Stopped schooling in kindergarten due

to illness Mother is a 35 year old housewife Father is a 53 year old construction

worker

Physical Examination Awake, alert, ambulatory with support,

NICRD BP 110/70, HR 110 bpm, RR 23 cpm, T

38oC Weight 15 kg, Height 109.5 cm, BMI 13,

WFA=71 (moderate PEM), HFA=95 (no stunting), WFH=83 (mild wasting)

Warm, moist skin, no active dermatoses

Physical Examination Pink palpebral conjunctivae,

anicteric sclerae (+) right auricular swelling,

(-) discharge, (-) tenderness (+) saddle nose deformity,

(-) discharge, (-) tenderness (-) oral ulcers, (-)

tonsillopharyngeal congestion trachea at midline, (-)

cervical lymphadenopathy

Physical Examination Equal chest expansion, (-) retractions, clear

breath sounds, (-) wheezes, (-) crackles Adynamic precordium, distinct heart sounds,

tachycardic, regular rhythm, (-) murmur, (-) heaves, (-) thrills

Flat, soft, normoactive bowel sounds, (-) tenderness, (-) organomegaly, LE edge not palpable, (-) masses

Grossly male, descended testes, (-) lesions, (-) discharge

Physical Examination Full and equal pulses (+) swelling and

tenderness of right elbow, bilateral wrists, left knee, left ankle, (+) superficial abrasion on left ankle, (+) linear scar over left ankle and left knee

(-) cyanosis, (-) jaundice

Physical ExaminationLeft knee: 0°-90°, Left ankle: dorsiflexion: 0° plantar flexion: 0°-

20° inversion: 0°-5° eversion: 0°Right ankle: full range

of motion

Physical ExaminationNeuro Exam General survey: Awake, coherent Cranial nerves: Pupils 2-3 mm EBRTL, brisk

corneals, (-) facial asymmetry, tongue midline, (+) gag reflex, good shoulder shrug

Sensory: 100% on all extremities Motor: 5/5 on all extremities DTRs: ++ on all extremities Cerebellar: Can do FTNT and APST with ease Meningeals: Supple neck, (-) nuchal rigidity Other reflexes: (-) Babinski

Initial Assessment

Possible Relapsing Polychondritis

Summary 6 year old male Admitted for swelling of the right ear Right ankle pain and swelling Swelling of the nose Swelling of the left ankle and left knee Progressive joint swelling of both wrists

and elbows

Arthritis

Arthritis Inflammation of a joint space associated

with joint swelling, pain, and limitation of motion

Results from infection, trauma, degenerative changes, or metabolic disturbances

Extra articular involvement with arthritis: Wegener’s Granulomatosis and Behcet’s Disease

Wegener’s Granulomatosis nasal/oral inflammation saddle nose deformity lung nodules/cavities microhematuria/red cell casts

Behcet’s Disease arthritis and arthralgia recurrent oral ulceration (3x per year) recurrent genital ulceration eye lesions skin lesions like erythema nodosum

Monoarticular Arthritis TB Arthritis responsive to Anti Koch’s

Septic Arthritis responsive to antibiotics acute onset

Polyarthritis

Gout excruciating, sudden,

unexpected, burning pain swelling, redness, warmth,

and stiffness involving ankle, heel, instep, knee, wrist, elbow, fingers, and spine

tophi affecting the big toe and helix of the ear

Osteochondroma involvement of cartilage involvement of bone enlarging mass weight loss

Juvenile Idiopathic Arthritis more than 6 weeks of pain,

swelling, and stiffness of joints involves metacarpophalangeal

joints, proximal interphalangeal joints wrists, and metatarsophalangyeal joints

involvement of cartilages such as the ears and nose

nonerosive and asymmetric

Relapsing Polychondritis

Definition Multisystemic disorder of unknown etiology

affecting young adults

Recurrent, progressive episodes of inflammation affecting the cartilaginous structures, resulting in tissue damage

Elastic cartilage of the ears and nose, hyaline cartilage of peripheral joints, fibrocartilage of the axial skeleton, and cartilage of the tracheobronchial tree

Diagnostic Criteria (3 or more) recurrent chondritis of both auricles

non erosive inflammatory polyarthritis

chondritis of nose cartilage

inflammation of ocular structures (keratitis, scleritis, episcleritis, uveitis)

chondritis of the respiratory tract (laryngeal and/or tracheal cartilages)

cochlear and/or vestibular damage causing sensorineural hearing loss, tinnitus and/or vertigo

Course in the Hospital

Rheumatologic Referred to Pediatric Rheumatology and

Orthopedics

Referred to Otorhinolaryngology for evaluation and anticipatory care for airway

Xrays: decreased joint space and osteochondral changes on the left knee and left ankle, no joint space, no osteochondral changes on the right ankle

Rheumatologic Diagnostic aspiration on the right ear

Neck STAPL: intact tracheobronchial airway and no obstruction

Rheumatologic Started on Prednisone 10 mg/tab (1.5 mkd) 1 tab

OD, Naproxen 275 mg/tab (20 mkd) ½ tab BID, Oxacillin (250) 750 mg/IV q6

2nd HD, (+) resolution of joint swelling and tenderness, afebrile, with good activity and appetite

P> Oxacillin shifted to Cloxacillin 250 mg/5 ml (120) 9 ml q6

Prednisone increased to 10 mg/5 ml (2 mkd) 5 ml TID

Cardiac Referred to Pediatric Cardiology for evaluation of

cardiovascular functioning

ECG: sinus tachycardia, no axis deviation, no chamber enlargement

2D Echo: fair LV systolic function, mild TR, LVE, no vegetation, minimal pericardial effusion

CK MB and Troponin I: positive

A> Possible Myocarditis vs Cardiomyopathy

Cardiac P> Started on Dobutamine (5 mcg/kg/min) at 5

cc/hr, Furosemide (1) 15 mg/IV OD, and Lanoxin (0.003) 0.25 mg/tab, 0.045 mg/pptab, 1 pptab q12

2nd HD, comfortable, not in acute distress with HR 90 bpm

Dobutamine discontinued, Lanoxin continued, and Furosemide was shifted to PO 20 mg/tab (0.75) ½ tab OD

Etiology Remains unknown

Loss of basophilic staining of the cartilage matrix with perichondral inflammation of the cartilage

Perivascular mononuclear and polymorphonuclear cell infiltrates

Chondrocytes become vacuolated, necrotic and replaced by fibrous tissue

Etiology Release of degradative enzymes

Immune mediated activation of chondrocytes and other inflammatory cells by cytokines including IL-1 and TNF-a

Autoimmunity

Prevalence 3.5 cases/million in the US (Doros, A.A,

October 2004)

4 cases seen in PGH

Peak age for disease onset is the 5th decade

Female preponderance with ratio of 3:1

Clinical Features Auricular chondritis

Joint pain with or without arthritis involving metacarpophalangeal, proximal interphalyngeal joints, wrists and knees

“Saddle nose” deformity

Scleritis, episcleritis, keratitis, and conjunctivitis

Clinical Features Hoarseness, non productive cough, dyspnea,

wheezing, and inspiratory stridor

Tenderness over thyroid cartilage and trachea

Aortic regurgitation and mitral regurgitation

Thoracic and abdominal aneurysm, myocarditis, pericarditis, silent myocardial infarction, paroxysmal atrial tachycardia, and 1st degree or even complete heart block

Clinical Features Mild mesangial proliferation, focal and

segmental necrotizing glomerulonephritis with crescents

Cranial nerve palsies, cerebral aneurysms and aseptic meningitis

Diagnostic Methods No specific diagnostic tools

Serum autoantibodies to collagen II and to 148 kDa noncollagenous cartilage matrix protein

Chest conventional radiographs, pulmonary function test, and chest CT tomography

Cartilage biopsy

Laboratory Findings Elevated ESR, CRP, anemia, leucocytosis, and

thrombocytosis

Serum antibodies to collagen II

Elevated urinary glycosaminoglycans levels

Serum 148 kDa noncollagenous matrix protein

(-) Rheumatoid factor, antinuclear antibodies or antineutrophil cytoplasmic antibodies

Management NSAIDs and Prednisone (1 mkday) Methotrexate Cyclophosphamide Anti CD4 monoclonal antibody Autologous stem cell transplantation Surgery: Tracheostomy

Prognosis Progressive disease

Intermittent or fluctuant inflammatory manifestations

Persistent symptoms between acute flares

Develops some degree of disability over the time

Overall survival rates were 74% at 5 years and 55% at 10 years

Updates on the Patient Swelling of ears and joints of other extremities Saddle nose deformity Cushingoid facies and hirsutism

Maintained on Prednisone, Lanoxin, Furosemide

2D Echo after 3-6 months Rehabilitation Hearing screen and Ophtha evaluation

Conclusion Correct and early diagnosis of polyarthritis are vital

Arthritis may be the initial clue to a serious systemic disease

Clinical diagnosis

Life threatening complications require awareness and recognition of disease

Long term follow up is necessary

Thank you!