Juvenile Spondyloarthritis and Fever Syndromes


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Juvenile Spondyloarthritis and Fever Syndromes by Reema Syed,MD, Assistant Professor of Internal Medicine and Pediatrics, Division of Adult and Pediatric Rheumatology, Saint Louis University

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Juvenile Spondyloarthritis and Fever Syndromes

  1. 1. Juvenile Spondyloarthritis Reema Syed,MDAssistant Professor of Internal Medicine and Pediatrics Division of Adult and Pediatric Rheumatology Saint Louis University
  2. 2. Objectives• Understand the classification of juvenile SpA• Understand treatments in Juvenile SpA
  3. 3. ILAR criteria for JIA• OligoJIA (1-4 joints, persistent or extended)• PolyJIA (5 or > ) Rheumatoid Factor -• PolyJIA Rheumatoid Factor +• Systemic onset JIA: fevers, rash, joint pain• Psoriatic Arthritis• Enthesitis related arthritis• Undifferentiated arthritis
  4. 4. Spondyloarthritis• Ankylosing spondylitis• Undifferentiated spondyloarthritis• Reactive arthritis• Arthritis associated with IBD• Psoriatic arthritis
  5. 5. Juvenile SpA• Often undifferentiated at onset• Less likely to affect axial skeleton• More likely to affect hips and peripheral enthesis (sites of ligament/tendon insertions)
  6. 6. Juvenile PsA• Arthritis plus psoriasis• OR• Arthritis + 2: – Dactylitis – Nail pitting or onycholysis – Ps in 1st degree relative
  7. 7. Enthesitis Related Arthritis• Arthritis + enthesitis• OR• Arthritis or enthesitis + 2 or more: – Presence of SI joint tenderness or inflammatory back pain – + HLAB27 – Onset of arthritis in male after 6year of age – Acute anterior uveitis – h/o AS, ERA, IBD related arthritis, reactive arthritis, acute anterior uveitis in 1st degree relative
  8. 8. Undifferentiated JIA• Arthritis that does not fullfill criteria in any of the other categories or fulfills criteria in 2 or more of the other categories
  9. 9. Juvenile AS• Term used to describe child < 16 with typical symptoms of AS – Bilateral sacroiliitis OR – Unilateral sacroiliitis• PLUS – Inflammatory back pain – Limitation of lumbar motion OR – Decreased chest wall expantion• Juvenile onset AS: symptoms of AS began prior to age 16yr but criteria met after 16 years
  10. 10. Clinical features associated with spondyloarthritis• Enthesitis: – Tenderness/ inflammation at point of tendonous/ ligamentous insertions – Inflammatory back pain (age of onset < 40yrs, improved with exercise, no improvement or worsening at rest, pain at night)• Alternating buttock pain• Dactylitis: sausage like swelling of 1 or more digits• Acute anterior uveitis: eye pain, redness, intolerance to light• IBD: ulcerative colitis, crohn disease• Nail pitting• Psoriasis
  11. 11. Genetic susceptibility of SpA• AS: HLAB27 40% heritability – Present in 90% of AS patients – Present in 60-90% of Juvenile AS – (ONLY 5% OF HLAB27 CARRIERS DEVELOP AS)• ERAP1• IL23R
  12. 12. Juvenile SpA: treatment• Sulfasalazine: beneficial for peripheral arthritis• NSAIDs• Anti-TNF biologic agents – Etanercept – Adalimumab – Remicade• Exercise• PT/OT• education
  13. 13. Juvenile SpA: outcomes• Predictors of failure to achieve remission – Genetic components: • HLADRB1 08 • FH of AS in 1st degree relative – Clinical features • Hip or ankle arthritis w/in the 1st 6 months of disease
  14. 14. Hereditary Autoinflammatory diseases: Periodic Fever & Cryopyrinopathies Reema Syed,MDAssistant Professor of Internal Medicine and Pediatrics Division of Adult and Pediatric Rheumatology Saint Louis University
  15. 15. Objectives• Understand the different classes of fever syndromes & cryopyrin associated periodic fevers syndromes (CAPS)• Understand the specific symptoms and complications of fever syndrome & CAPS• Understand therapies available for specific fever syndromes & CAPS
  16. 16. Periodic Fevers• Familial mediteranean fever (FMF)• Mevalonate kinase deficiency/ hyper Immunoglobulin D syndrome (MKD/ HIDS)• TNF receptor associated periodic syndrome (TRAPS)• NALP12 associated periodic fever (FCAS2)
  17. 17. CAPS• Familial Cold Autoinflammatory Syndrome (FCAS)• Muckle Wells Syndrome (MWS)• Neonatal Onset Multisystem inflammatory disease (NOMID)/ Chronic Infantile Neurologic Cutaneous Articular (CINCA) syndrome
  18. 18. FMF• Most common periodic fever• Most commonly seen in the eastern mediterranean region• Recurring fever > 38 degrees – Lasting 6hrs to 3 days – Frequency: every week to every 3-4 months• Abdominal pain• Chest pain• Joint swelling/ pain• Rash (legs)
  19. 19. FMF• Treatment – Colchicine – Kineret (IL-1 receptor antagonist) – NSAIDs – Interferon alpha – Steroids
  20. 20. FMF• Complication – Amyloidosis
  21. 21. HIDS• Begins in 1st year of life-early childhood• Most common in western Europeans• Abrupt irregular attacks• Lasts 3-7day• High fevers• Neck pain• Enlarged lymph nodes• Belly pain, diarrhea, vomiting• Joint pain• Rash• Ulcers (oral, genital)
  22. 22. HIDS• Treatment: – NSAIDs – Kineret – Etanercept
  23. 23. HIDS• Complications – Mevalonic aciduria: CNS problems, eye problems, growth/ weight abnormalities
  24. 24. TRAPS• Age of onset: infancy to 50years• Recurrent fevers (2-6 episodes a year)• Fevers lasting 3-4 weeks• Belly pain• Joint pain• Diarrhea• Muscle pain• Rash• Conjunctivitis
  25. 25. TRAPS• Treatment: – Steroids – Etanercept – kineret• Complication: – amyloidosis
  27. 27. FCAS• Begins in infancy• Episodes last < 24hrs• Rash (related to cold exposure)• Conjunctivitis• Joint pain/ stiffness• Fever spikes (related to cold) with sweating• Overwhelming fatigue
  28. 28. MWS• Begins in infancy-adolescent years• Rash: hives• Joint pain continuous• Fatigue• Fevers• Conjunctivitis
  29. 29. MWS• Complication: – Amyloidosis
  30. 30. NOMID/CINCA• Appears in first 2 months of life• Symptoms are continuous• Rash: hive like• Hearing loss• Loss of vision• Deforming joint arthritis• Recurrent fevers• Facial bone deformities
  31. 31. NOMID/CINCA• Complications: – Amyloidosis – Bony deformities – Increased pressures in the skull
  32. 32. CAPS treatment• Kineret• Rilonacept• canakinumab