Juvenile Idiopathic Arthritis (JIA)

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1 Juvenile Idiopathic Arthritis (JIA) Kaveh Ardalan, MD, MS Division of Rheumatology Ann & Robert H. Lurie Children s Hospital of Chicago Assistant Professor, Pediatrics and Medical Social Sciences Northwestern University Feinberg School of Medicine *Contents adapted from AAP slide deck provided by MSK bootcamp organizers, credit Elizabeth Kessler, MD, MS, Children s Mercy Kansas City

2 I have no relevant financial relationships with the manufacturers of any commercial products and or providers of commercial services discussed in this CME activity I do not intend to discuss an unapproved or investigative use of a commercial product/device in my presentation Only other disclosure: travel reimbursement for a single day biomarker/trial design workshop sponsored by Reveragen

3 Objectives Know the definition of arthritis Recognize the subtypes of juvenile idiopathic arthritis (JIA) Know when to order and how to interpret common rheumatologic labs in a child with joint pain

4 Etiology of Joint Complaints in Childhood Infection Post-infectious/Reactive Orthopedic/Trauma Rheumatic diseases Hematologic Genetic/metabolic/ Malignancy Immunodeficiency Psychogenic/Behavioral

5 Arthritis: Definition Objective signs of inflammation within the joint Swelling or an effusion OR Limitation in range of motion plus: Tenderness to palpation (small joints) Pain with movement OR Petty et al, J Rheumatol Feb;31(2):390-2.

7 Inflammatory Arthritis Pain (Arthralgia) Arthritis Arthritis is: Inflammation of the joints. Characterized by swelling, warmth, redness, stiffness, decreased range of motion, pain. Multiple causes: infection, trauma, systemic autoimmune disease, idiopathic Synovium Cartilage NORMAL JIA Inflamed joint lining (synovium) Synovial fluid

8 Joint Complaints: History Is the complaint mechanical or inflammatory? Mechanical: no AM stiffness, pain with activity, improves with rest, no or minimal swelling Inflammatory: AM stiffness, pain improves with activity, associated with swelling, warmth Arthritis!

9 Juvenile Idiopathic Arthritis (JIA) Heterogeneous group of inflammatory disorders Characterized by chronic arthritis Clinical diagnosis

10 Definition of JIA 1. Arthritis in one or more joints for at least 6 weeks Swelling or effusion Limited Range of Motion plus either: Tenderness to palpation OR Pain with Range of Motion 2. Age of onset <16 years 3. Diagnosis of exclusion- other identifiable causes have been ruled out Petty et al, J Rheumatol Feb;31(2):390-2.

11 Indolent onset Symptoms of JIA Stiffness, especially in AM or after a sedentary period Limping or reluctance to walk Difficulty with buttons, snaps, zippers, opening jars and bottles, writing Improves as day progresses or with activity Discomfort without severe or acute pain Swelling (not always noticed)

12 Symptoms of JIA McGhee et al. Pediatrics 2002 Isolated MSK pain without other signs or symptoms is almost never the presenting complaint of chronic arthritis in kids JIA usually presents as joint swelling or limp Children referred with +ANA and/or RF were no more likely to have a chronic inflammatory disease than those who did not include such results as a reason for referral. McGhee et al., Pediatrics Aug;110:354-9

13 History of JIA Classification JRA (1977) JCA (1978) JIA (1997) Pauciarticular Pauciarticular Oligoarticular Persistent Extended Systemic Systemic Systemic Juvenile AS Psoriatic Polyarticular Polyarticular Polyarticular RF+ RF- Enthesitisrelated Psoriatic Undifferentiated

14 Epidemiology Incidence ~10-20/100,000 children Prevalence at least 100/100,000 children Varies by subtype and ethnicity

15 JIA Subtypes Oligoarticular (<5 joints), about 50% Persistent or Extended Polyarticular ( 5 joints) RF negative or positive Systemic Psoriatic Enthesitis Related/Spondyloarthropathy Undifferentiated

16 Oligoarticular JIA Most common form Girls: Boys 3-5:1, peak age 1-4 years Northern European Most common presentation: single swollen knee Limp, worse in AM or after nap May not complain of any pain Otherwise well-appearing

17 Rheumatology Image Library. American College of Rheumatology

18 Complications Flexion Contracture Leg Length Discrepancy

19 Complications Flexion Contracture Leg Length Discrepancy Atrophy Rheumatology Image Library. American College of Rheumatology

20 Uveitis and JIA Usually asymptomatic until damage occurs Chronic in most subtypes Risk factors: younger age, +ANA Up to 20% of oligoarticular JIA Complications: cataracts, glaucoma, blindness Rheumatology Image Library. American College of Rheumatology

21 Recommended Frequency of Ocular Examinations Pediatrics 117: , 2006

22 When do I draw antibodies to diagnose arthritis? NEVER-Arthritis is a clinical diagnosis! Rheumatoid Factor (RF): Negative in 95% of children with arthritis. Positive in many conditions including infections, lupus, liver disease, and malignancy Most common cause of + RF in children is viral infection

23 When do I draw antibodies to diagnose arthritis? Antinuclear Antibody (ANA): Does NOT help with diagnosis of JIA, only to determine risk of uveitis Sensitive for SLE only Positive in liver disease, infections, malignancy, and well children Only 5% of +ANA tests are due to lupus many are in healthy people

24 Polyarticular JIA Girls: Boys 3:1 2 age peaks: Age 1-3: RF negative Later childhood/ adolescence: RF positive Symmetrical, small joints, C-spine, TMJ disease +/- mild systemic disease: anemia, fatigue, ESR More aggressive, requires more medication

25 Symmetrical hand and wrist arthritis in Poly JIA Rheumatology Image Library. American College of Rheumatology

26 C-Spine and TMJ Arthritis

27 TMJs and C-spine Often Involved

28 Spondyloarthropathies Enthesitis Related Arhritis Psoriatic Arthritis Reactive Arthritis Ankylosing Spondylitis Undifferentiated Spondyloarthropathy IBD Associated Arthritis

29 JIA Subtypes Oligoarticular (<5 joints), about 50% Persistent or Extended Polyarticular ( 5 joints) RF negative or positive Systemic Psoriatic Enthesitis Related/Spondyloarthropathy Undifferentiated

30 Common Features Peripheral (esp. knees, hips and ankles), sacroiliac and spine involvement potentially Enthesopathy is common (Achilles, patellar) Sausage digits or Dactylitis Older age, Boys > Girls Acute anterior uveitis HLA B27-related

31 Enthesitis

32 Dactylitis ( Sausage Digit ) Rheumatology Image Library. American College of Rheumatology

33 Inflammatory Eye Disease Conjunctivitis Transient Anterior Uveitis Acute onset, unilateral>bilateral, painful, red eye Can cause permanent vision loss if not treated Does not always parallel arthritis activity

Psoriatic Arthritis Often precedes the onset of psoriasis in children Clues to diagnosis: 1 st degree relative with psoriasis Nail pitting DIP

35 Psoriatic Arthritis Often precedes the onset of psoriasis in children Clues to diagnosis: 1 st degree relative with psoriasis Nail pitting DIP involvement (33%) Dactylitis (sausage digit) Small joints hands/feet Aggressive, erosive disease Rheumatology Image Library. American College of Rheumatology

36 Psoriatic Arthritis (PsA) Plaque psoriasis (psoriasis vulgaris) is most common skin phenotype in PsA 15% develop psoriasis AFTER onset of arthritis Often within 2 years in kids Some pts may be unaware they have it Scalp, behind ears, navel, nail changes Little relationship between skin severity and arthritis severity

37 Skin Findings Rheumatology Image Library. American College of Rheumatology

38 Enthesitis-Related Arthritis Male > Female Strong association with HLA-B27 Arthritis and enthesitis OR: Arthritis or enthesitis with 2 of the following: SI joint tenderness, +HLA-B27, arthritis in a boy 6 years of age or older, acute anterior uveitis, or 1 st degree relative with spondyloarthritis Peripheral axial disease

39 Systemic Onset JIA: An autoinflammatory disease Clinical Manifestations: Arthritis (may be absent at onset) High grade spiking fever Evanescent rash +/- Adenopathy +/- Hepatosplenomegaly +/- Pleural/pericardial effusion Rheumatology Image Library. American College of Rheumatology

40 Quotidian Fever Evanescent Rash Rheumatology Image Library. American College of Rheumatology

41 Differential Dx of SOJIA Primarily a diagnosis of exclusion Infection Inflammatory bowel disease Malignancy Connective tissue disease - SLE, JDM, PAN Acute Rheumatic Fever Kawasaki s disease

42 SOJIA Prognosis Past: steroid dependant toxicity/ growth failure Current: More targeted therapy: IL-1 and IL-6 blockade Rheumatology Image Library. American College of Rheumatology

Increased recruitment and activation of macrophages leads to tissue damage and organ failure

43 Macrophage Activation Syndrome (MAS) A life-threatening complication of SOJIA Excessive/Unregulated expansion of Macrophages/ T cells produces a cytokine storm. Increased recruitment and activation of macrophages leads to tissue damage and organ failure Cassidy s textbook of Pediatric Rheumatology Activated macrophage with hemosiderin deposits and a degenerating phagocytosed nucleated cell

44 MAS Acutely ill with purpura, mucosal bleeding Sustained fever Hepatosplenomegaly, adenopathy CNS dysfunction Lethargy with irritability, disorientation, headache, seizures, coma Liver dysfunction: AST, ALT, PT, PTT elevated Pancytopenia Low ESR due to low fibrinogen and liver dysfunction.

45 JIA Treatment Goals of treatment: control inflammation to prevent damage: erosions, contractures, deformities. Once there is damage we have little power to reverse it.

46 JIA Treatment Medications NSAIDS for mild disease Systemic Steroids: try to avoid, do not affect long term outcome Intra-articular steroids Disease modifying agents (DMARDS): methotrexate Biologics: target specific inflammatory mediators TNF alpha, IL-1, IL-6, Tcell costimulatory molecules, others

47 Changes to consider in practice A careful history and exam >> labs in diagnosing arthritis. ANA and RF are prognostic, not diagnostic, tests in JIA. Avoid starting systemic steroids if you do not know the diagnosis.

48 References Petty et al. International League of Associations for Rheumatology Classification of Juvenile Idiopathic Arthritis: Second Revision. J Rheumatol Feb;31(2): McGhee et al. 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 Aug;110:354-9 Rheumatology Image Library. American College of Rheumatology Cassidy et al. Ophthalmologic examinations in children with juvenile rheumatoid arthritis. Pediatrics May;117(5): McGonagle, D. Arthritis Rheum.1999; 42:

Department of Paediatrics Clinical Guideline. Guideline for the child with possible arthritis (joint swelling/pain, loss of function)

Department of Paediatrics Clinical Guideline Guideline for the child with possible arthritis (joint swelling/pain, loss of function) Definition: Juvenile Idiopathic Arthritis (JIA) is defined as arthritis