Board Review: Pediatric Rheumatology

1. Board Review: Pediatric Rheumatology Kathy Haines, MDJennifer Weiss, MD Chief of Pediatric Rheumatology Joseph M. Sanzari Children’s Hospital Hackensack University Medical Center Associate Professor of Pediatrics UMDNJ-New Jersey Medical School

2. Arthritis • More than 100 causes of arthritis in children • Arthritis is common in rheumatic diseases • But not all rheumatic diseases are associated with arthritis

3. Acute Causes of Arthritis • Traumatic • Hemarthrosis • Infectious • Bacterial (septic arthritis, osteomyelitis, Lyme) • Viral (esp rubella, parvo) • Post-Infectious • Reactive arthritis • Toxic/transient synovitis • Allergy • Serum sickness • Acute Inflammatory and Rheumatic Conditions • HSP • Kawasaki disease • Rheumatic fever

4. Most Common Causes of Chronic Arthritis in Children • Juvenile Idiopathic Arthritis • What we used to call “JRA” PLUS • Juvenile Spondyloarthritis • Juvenile Psoriatic Arthritis • Other chronic rheumatic syndromes • SLE • Dermatomyositis • Vasculitis

5. Other causes of joint pain:Mimics of arthritis • Mechanical abnormalities • Patellofemoral Syndrome • Hypermobility Syndromes • Malignancies • Endocrine abnormalities • Inherited bony dysplasias • Chronic pain syndromes • Psychogenic causes

6. Acute Rheumatic Syndromes Reactive arthritis Transient/toxic synovitis HenochSchonlein Purpura Kawasaki Disease Acute Rheumatic Fever

7. Reactive Arthritis(aka Reiter Syndrome) • Arthritis and other extra-articular features following infections • Classical triggers: • Enteric (Salmonella, Shigella, Yersinia) • Non-gonoccoal urethritis • Other infections commonly cause reactive arthritis • Varicella • Parvovirus • Group A Streptococcus (rheumatic fever and post-strep reactive arthritis) • Many other nonspecific infections • Extra-articular features: • Conjunctivitis or uveitis • Urethritis • Rash (keratoderma blenorrhagicum) • Not common in children

8. Transient synovitis • Acute hip synovitis in toddlers-early school age • Often follows onset of URI • Usually unilateral leg (hip) pain • May have low grade fever • Labs usually normal • PE: Pain and limitation of motion of affected hip • Self-limited: resolves within a few days to weeks

9. HenochSchonlein Purpura • Common acute vasculitis of childhood • Manifestations: • Purpura • Usually limited to lower extremities and buttocks • Arthritis • Nephritis • Abdominal pain • Usually self-limited (resolves after a few weeks) • Often post-infectious (especially Strep)

10. HSP

12. HSP continued • Purpuric rash on the LE is almost pathognomonic (in the absence of infection or coagulopathy) • Biopsy: • Leukocytoclastic vasculitis (seen in many other types of vasculitis) • IgA deposits on IF (diagnostic for HSP) • Nephritis: • IgA nephropathy • Usually manifests as hematuria which resolves • Need to check urinalysis frequently (weekly) for a month and then monthly • Some develop chronic renal disease

13. HSP Treatment • Symptomatic • NSAIDs (joints) • Steroids (reserved for significant GI involvement)

14. Kawasaki Disease • Most common vasculitis in young children • Usually 6 years or younger • Criteria (5 of 6 must be present for definite dx) • High grade fever for more than 5 days • Polymorphous rash (MP, scarlatiniform, morbilliform), often starts in the groin and diaper area • Non exudative conjunctivitis • Cervical lymphadenopathy >1.5 cm (usually unilateral) • Mucositis (strawberry tongue, red lips and mouth, vertical cracking of lips) • Extremity changes (red palms and soles, edema of hands and feet)

15. KD conjunctivitis

16. Polymorphous rash of KD

17. Typical mucositis of KD

18. Unilateral lymphadenopathy

19. Acute Extremity Changes of KD

20. Late extremity manifestations of KD

21. Kawasaki Disease: Labs • Labs reflect highly inflammatory state • Elevated WBC, ESR, CRP • Thrombocytosis comes later (1 to 2 wks after onset) • Elevated LFTs (transaminases) common • Sterile pyuria • Aseptic meningitis • No specific diagnostic test

22. Kawasaki disease treatment • Treatment • IVIG (2gm/KG): best outcome if prior to 10 days of fever • Low dose aspirin • Other (for refractory cases): • Pulse steroids • Anti-TNF therapy • Complications: • Coronary artery aneurysms • Myocarditis • Myocardial infarction • Atypical KD patients that do not fulfill criteria is not uncommon • Fever, rash, conjunctivitis most common features

23. Acute Rheumatic Fever • Post-Streptococcal rheumatic syndrome • Fever (usually low grade) and migratory polyarthritis 1-2 weeks following Strep infection (which can be subclinical) • Carditis is common but not always present • Mitral insufficiency most common • Acute phase reactants (ESR and CRP) always elevated

24. ARF Jones Criteria • 2 Major criteria, or 1 Major and 2 minor criteria • Major • Migratory polyarthritis • Carditis (valvulitis, especially mitral) • Erythema marginatum • Subcutaneous nodules • Chorea • Minor • Fever • Elevated ESR • Arthralgias (doesn’t count if has polyarthritis as major) • Prolonged PR interval Required: Evidence of recent Strep infection

25. Uncommon Manifestations of ARF: Nodules and erythema marginatum

26. Treatment of Acute Rheumatic Fever • Aspirin or NSAIDs • The arthritis will “melt away” • Must be taken consistently until ESR normalizes • Steroids reserved for severe carditis • Penicillin prophylaxis (until adulthood?) • PO • Monthly IM bicillin

27. A 6 year old boy has a 24 hr history of fever, malaise, and bruising. He appears ill and his temperature is 103. He has widespread petechiae and palpable purpura on the buttocks and lower extremities. The hgb is 10.5, WBC 22.5, and platelets are 25,000. The most likely diagnosis is: • Idiopathic thrombocytopenic purpura • HSP • Leukemia • Meningococcemia • Rocky Mountain spotted fever

28. An 8 year old girl has a painful left knee. Two days ago her right ankle was painful and swollen, but today it seems fine. She has a fever to 101, and her resting heart rate is 120. Her knee is swollen and painful, but her ankle is completely normal. She has a Grade 3/6 systolic murmur. The most likely diagnosis is: • Dermatomyositis • Systemic JIA • Acute rheumatic fever • Septic arthritis • Systemic lupus erythematosus

29. A 5 year old boy has been limping for 3 days. He had an URI earlier in the week and there is no hx of trauma. His temp is 99.9, and he is limping. There is pain with flexion and internal rotation of his right hip. The WBC is 4.5 and ESR is 20. The most likely diagnosis is: • Legg Perthes disease • Slipped capitol femoral epiphysis • Oligoarticular JIA • Septic arthritis • Transient synovitis

30. Chronic Arthritis in Children

31. Chronic Arthritis in Children(JRA and JIA) • Pauciarticular JRA / Oligoarticular JIA • Polyarticular JRA / Polyarticular JIA • RF positive • RF negative • Systemic JRA / Systemic JIA • Spondyloarthropathies / Enthesitis related JIA • Psoriatic arthritis / Psoriatic JIA

32. JRA/JIA Definition • Must have had arthritis in at least one joint for > 6 weeks • Must be less than 16 years old at onset of symptoms • Must exclude all other conditions • No tests are diagnostic

33. Pauciarticular JRAOligoarticular JIA • Definition • Four or less joints • Usually little girls (average age: 2 yrs) • Insidious onset of a swollen joint, most often the knee • Labs usually normal except: • ESR may be mildly elevated • ANA often positive • High risk of iritis/uveitis

34. Uveitis in JIA

35. Bone deformities, contractures& atrophy

36. Polyarticular JRAPolyarticular JIA • Definition: • 5 or more joints involved • No systemic sx • RF serologic status is important in classification • RF negative poly JIA (young girls: mean age 4) • RF positive poly JIA (older pre- to teen aged girls >10 years)

37. RF Negative Polyarticular JIA • >5 joints, but usually has symmetrical arthritis in many joints, including small joints • RF negative • ANA + in 50% or more • Elevated ESR, CRP, Ig’s • Usually younger girls than RF positive • Moderate risk for uveitis

39. RF Positive Polyarticular JIA • Positive rheumatoid factor test • Identical to adult Rheumatoid Arthritis • Pre-teen/teen onset most frequent • Similar joint pattern to RF negative polyarthritis • Elevated ESR, CRP • Hallmark: rheumatoid nodules

40. Systemic JRA or JIA • Fevers • High (>39) • Quotidian pattern is common • Rash • Non-fixed pink eruption • Worsens with fever spike • Koebner phenomenon • Arthritis (any number of joints can be affected) • Other features: • Generalized lymphadenopathy • Hepato- or splenomegaly • Serositis (Pleuritis or pericarditis)

42. Quotidian Fever Pattern

43. Systemic JIA • Laboratory features • High WBC (poly predominant) • Anemia common • Thrombocytosis (often 5-800,000) • Very high ESR and CRP • Extremely high ferritin levels (500-10,000+) • Negative serologies • Other complications • Tamponade • Macrophage activation syndrome (can be fatal) • Pulmonary hypertension • Uveitis is not seen

44. Juvenile SpondylarthropathiesEnthesitis related JIA • Arthritis and enthesitis (usually calcaneal) • More common in pre-teens and teens • Boys > girls • Other features • HLA B-27 commonly positive • Acute anterior uveitis • Sacroiliitis • Inflammatory spine pain and limitation of flexion • Positive family history: anterior uveitis, spondylarthropathies, especially ankylosing spondylitis, Reiter syndrome and inflammatory bowel disease

45. Spondylarthropathy / Enthesitis related JIA Features

46. Juvenile Psoriatic ArthritisPsoriatic JIA • Arthritis and psoriasis OR • Arthritis and a positive family history of psoriasis plus • dactylitis • nail pitting or onycholysis

47. Psoriatic arthritis features

48. Psoriatic arthritis • Can mimic any of the following: • Oligoarticular JIA • Polyarticular JIA • Enthesitis related JIA and ankylosing spondylitis • Dactylitis (isolated swollen digits) is the most specific type of arthritis associated with arthritis

49. Dactylitis in psoriatic arthritis

50. JIA Treatment in a nutshell • Oligoarticular forms of JIA • NSAIDs • Intra-articular steroids • Polyarticular forms of JIA • NSAIDs • Methotrexate • Prednisone is not usually needed • Anti-TNF biologics • Systemic JIA • NSAIDs • Steroids for severe systemic symptoms (especially pericarditis) • Methotrexate for arthritis • Anti-IL1 and IL6 biologics