pyrexia of unknown origin? think still · case presentation •fm, 4 years old female. admitted on...
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Pyrexia of unknown origin? Think still
Lawrence Owino Okong’o,
Mmed (UoN); Mphil. (UCT).
Lecturer, Department of Paediatrics and Child Health,
University of Nairobi. Paediatrician/ Rheumatologist.
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DISCLAIMER
The presentation has been supported by ROCHE.
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Case presentation
• FM, 4 years old Female. Admitted on 12-2-2018 with 6 months history of: • General malaise,
• Pain and swelling of knee, ankle, elbow joints
• Recurrent fever.
• Admitted at a peripheral facility 1 month earlier with febrile illness and treated for malaria and antibiotics (ceftriaxone) with minimal improvement.
• FSH: Older of 2 siblings, other (male) alive and well. • Family history unremarkable.
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Admission physical exam findings
• Febrile T= 37.6C; Sick looking. Generalized lymphadenopathy 1-2cm, discrete. Mild pallor.
• Abdomen: generalized distension. Liver 6cm BCM.
• RS: Tarchypnoeic RR= 36; With flaring alar nasi and lower chest indrawing. Vessicular breath sounds. No added sounds and good air entry.
• ENT: No Ear or pharyngeal/ tonsillar inflammation.
• CVS: Tarchycardia 124/min. Normal heart sounds/ no added sounds.
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INVESTIGATIONS
• Blood , urine, stool, CSF cultures – negative.
• HIV? – Negative
• In view of persistent fever spikes and arthritis? • Rickettsia and Brucella serology: both negative.
• Malaria antigen test and film: negative.
• Leishmania IgG (21/3/18) = Neg
• ASOT was +; but RF negative.
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Tuberculosis or no tuberculosis?
• Mantoux – Negative (0 mm).
• Quantiferon: positive
• LN Biopsy: reactive. No granuloma. Negative ZN for AAFBs.
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Laboratory investigations
23/3/18 16-3-18 10/3/18 4/3/18 16/2/18
CRP(<10) 24 244 59.6 0
WBC 15.1 43 32
Hb 7.3 4.9 8.1
Plt (150-450) 481 573 369
Neutr 68.8%
ESR (0-20) mm/hr 80 80
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Laboratory investigations
Date 1/3/18
Rapid salmonella Ag Neg
ASOT (21/2/18) Neg
LNode Dermatopathic changes. No granuloma, no malignancy. ZN negative for AAFBs.
Blood, urine, stool, CSF M/C/S Repeatedly negative
BMA (21/2/18) No features of malignancy.
INR 1.38 (0.9-1.3)
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Working diagnoses
• 1. Septicemia
• 2. Tuberculosis.
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TREATMENT
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Rheumatology review 10/3/2018
• Noted history of fever on and off x2/12; alternate with periods of normal temp.
• Child very sick during spikes and generally well in between.
• History of associated joint pain and swelling.
• Had erythematous pruritic rash, most notable at height of fever spikes. Managed previously as allergy.
• Exam: Irritable, afebrile. Generalized lymphadenopathy.
• Abdomen distended with liver 6cm and spleen 2cm BCM.
• MSS: mild-mod wasting. Small effusion right knee.
• Skin: Generalized erythematous rash with scaling/ desquamation. No gottron’s, no malar rash.
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Progress
• During admission, developed severe anaemia and required transfusion in both instances.
• Post discharge: • The child continued to run daily fevers and complained of knee pain.
• Two weeks after discharge noted recurrence of the erythematous rash, generalized in face, trunk and limbs.
• Examination revealed slight knee effusion.
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Laboratory investigations
10/4/18 23/3/18 16-3-18 10/3/18 4/3/18 16/2/18
CRP(<10) 24 244 59.6 0
WBC 16.55 15.1 43 32
Hb 9.9 7.3 4.9 8.1
Plt (150-450) 582 481 573 369
Neutr 63.8 68.8%
LDH (125-220) 1017 4880
Ferritin (11-300 ng/ml) 1140 40405 <1200
Uric acid (149-506umol/l) 458 608 551
ESR (0-20) mm/hr 62 80 80
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Impression: Systemic JIA
• Based on: • quotidian fever, • rash, • arthritis, • poor response to
antibiotics, • hepatosplenomegaly, • lymphadenopathy.
• Investigations: • ESR, CRP raised. • Ferritin markedly raised. • LDH, Uric acid elevated.
• Cardiology review and repeat ECHO – normal CVS exam.
• Repeat evaluation by haematologist in view of anaemia and high Uric/LDH: • No features suggestive of
malignancy.
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Treatment
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SYSTEMIC JIA
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Introduction
• Juvenile Idiopathic Arthritis (JIA) refers to a heterogeneous group of chronic inflammatory diseases that share the common feature of arthritis of unknown origin occurring before the age of sixteen.
• JIA is the most common chronic rheumatic disease of childhood with prevalence estimated at 1:1000 for children under 16 years old.
J Rheumatol. 2004 Feb;31(2):390-2.
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JIA SUBTYPES
OLIGO <4 joints ANA Uveitis
Extended >5
6 months
ERA HLAB27
Psoriatic
Polyarthritis RF negative
Polyarthritis RF positive
Systemic JIA
other
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Pathogenesis
SoJIA Oligo/PolyArthritis ERA
Serology
RF Neg +ve in <25%
ANA Neg +ve in 50-85%
Genetics Polymorhisms in genes coding IL6, Macrophage inhibitory factor.
HLA associations observed: Poly - HLA-DR4 Oligo - HLA-A, HLA-B, & HLA-DR
HLA B27 +ve 80-90% pts with ank spondylitis. Polymorphisms in TNF, IL1 genes.
Environment Seasonal pattern suggests viral aetiology.
Infectious agents esp virus may play a role.
May be triggered by infectious in the GIT and GUT.
Int J Inflam. 2012;2012:271569, Orthop Clin North Am. 2012, http://www.pathophys.org/jia/.
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SoJIA: Back ground, epidemiology and pathogenesis
• Is a type of JIA associated with systemic inflammation.
• It is currently considered to be an autoinflammatory syndrome.
• The disorder was named after Sir George Frederic Still who first described it in 1896.
• Pathogenesis of SoJIA: • Triggers are unknown but infections suspected. • Contrasted to autoimmune diseases, in auto-inflammation, monocytes and
neutrophils rather than lymphocytes are the main effector cells. • They manifest with recurrent fever and inflammation in joints, skin, GIT, eyes. • They are associated with gene mutations with SoJIA associated with
polymorphisms in genes encoding IL-6, IL-10, TNF and IL-1.
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SoJIA2: Back ground, epidemiology and pathogenesis • Innate immunity cells such as monocytes and neutrophils are
expanded in number.
• There is also upregulation of innate immunity receptors such as TLR-5.
• JIA prevalence is estimated at 1:1000 for children under 16 years old; with SoJIA accounting for about 10% (30-50% in some series).
• In our setting, SoJIA accounts for about 16% of JIA cases.** (unpublished)
Mellins et al. Nat Rev Rheumatol. 2011
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SoJIA
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SoJIA Diagnostic criteria - ILAR
ILAR CRITERIA Our patient
1. Fever of at least 2 weeks plus
2. Arthritis in at least one joint plus
3. One or more of the following
Erythematous rash.
Generalized lymph node enlargement.
Hepatomegally/ splenomegaly.
Serositis.
4. Exclusions
psoriasis, presence of RF.
Ank. spondylitis, enthesitis or Arthritis in HLA B27+ male > 6 yrs
Petty et al. J Rheumatol. 2004
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sJIA: Quotidian fever, Rash
Photo Courtesy Prof. Scott rheumatology database UCT
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Treatment and Outcome of SoJIA.
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Ringold et al. ARTHRITIS & RHEUMATISM, October 2013,
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Outcomes 1: JIA is a chronic disease
Oen et al, J Rheumatol 2002
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Outcome 2: When do we stop therapy?
Criteria for inactive disease • NO joints with active arthritis • NO fever, rash, serositis. • NO active uveitis. • Normal CRP and ESR. • NO disease activity on the physician's global assessment of disease
activity • Clinical remission on medication: inactive disease =/>6 months while on
medication. • Clinical remission off medication: inactive disease =/>12 months while
the patient is off all medication.
Ravelli. Clin Exp Rheumatol 2006
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Outcome 3: Disease and treatment related damage
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MACROPHAGE ACTIVATION SYNDROME (MAS) ASSOCIATED WITH SoJIA
• MAS is an acquired form of hemophagocytic lymphohistiocytosis (HLH).
• MAS is a potentially fatal condition that is characterized by: • persistent fever, …. (CF Quotidian fever of SoJIA).
• Cytopenias ……. (Falling Hb, Falling Platelets, Falling ESR).
• liver abnormalities …. (Including very high ferritin),
• coagulopathy and
• central nervous system dysfunction.
• Well-differentiated macrophages with hemophagocytic activity are the pathologic hallmark of MAS (these may be seen in splenic aspirates/ BMA)
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MAS ASSOCIATED WITH SoJIA
• Approximately 10% of patients with sJIA are diagnosed with MAS.
• However, in as many as 50% of patients with active sJIA, MAS is evident in bone marrow aspirates
• Management of HLH:
MAS classification criteria
Ferritin ng/ml >684 And any 1 of:
Platelet u/L =<181
AST u/L >48
TGs mg/dl >156
Fibrinogen mg/dl >360
Ravelli Arthritis & rheumatology 2016
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SoJIA Management in Kenya
• Access to biologics is limited.
• Most of our patients thus have to contend with the relatively high adverse effect profile of NSAIDs and steroids.
• However, IL-6 receptor blockers (Actemra) is locally available and may be accessed in Kenyatta National Hospital.
• IL1 antagonist Anakinra is not yet available locally.
• There is some support from NHIF for biologics if appropriately motivated for.
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Your QUESTIONS
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My Quizz
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Anti-nuclear Abs are positive in most children
with systemic JIA. True or false?
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False, It’s auto-inflammatory.
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Rheumatoid factor is positive in most children
with systemic JIA. True or false?
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False, It’s auto-inflammatory not
autoimmune.
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Evanescent rash of SoJIA is rarely observed in
Africans due to dark skin. True or false?
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False, It’s often easily visualized
especially at the height of fever. And most Africans are brown
anyway!!.
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Which wrist is
affected in this child with JIA?
Why?
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Right. Increased bone age in affected joint due to
inflammation (vascular proliferation)
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Biologic therapy for systemic JIA targets which
two cytokines? Name the biologics.
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IL-1: Anakinra, IL-6: Tocilizumab (Actemra)
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Falling ESR and Platelets may be an indicator of good response
to therapy in children with systemic JIA. True or false?
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True. However if associated with
rising Ferritin, stabilization of rash and fever, liver and CNS
dysfunction then it may be indicative of MAS.
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Summary
• SoJIA is a subtype of JIA manifesting with quotidian fever, evanescent rash, arthritis and other systemic manifestations.
• It is an important differential in a patient with fever for whom an infectious origin has not been found.
• Safe and efficacious treatment agents including biologics exist for disease control.
• Untreated, the disease manifests severe early articular damage and complications such as MAS and anaemia.
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THANK YOU FOR GRACING THIS SESSION.