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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  • 1. Juvenile Spondyloarthritis Reema Syed,MDAssistant Professor of Internal Medicine and Pediatrics Division of Adult and Pediatric Rheumatology Saint Louis University
  • 2. Objectives Understand the classification of juvenile SpA Understand treatments in Juvenile SpA
  • 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. Spondyloarthritis Ankylosing spondylitis Undifferentiated spondyloarthritis Reactive arthritis Arthritis associated with IBD Psoriatic arthritis
  • 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. Juvenile PsA Arthritis plus psoriasis OR Arthritis + 2: Dactylitis Nail pitting or onycholysis Ps in 1st degree relative
  • 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. 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. 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. 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. 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. Juvenile SpA: treatment Sulfasalazine: beneficial for peripheral arthritis NSAIDs Anti-TNF biologic agents Etanercept Adalimumab Remicade Exercise PT/OT education
  • 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. 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. 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. 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. CAPS Familial Cold Autoinflammatory Syndrome (FCAS) Muckle Wells Syndrome (MWS) Neonatal Onset Multisystem inflammatory disease (NOMID)/ Chronic Infantile Neurologic Cutaneous Articular (CINCA) syndrome
  • 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. FMF Treatment Colchicine Kineret (IL-1 receptor antagonist) NSAIDs Interferon alpha Steroids
  • 20. FMF Complication Amyloidosis
  • 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. HIDS Treatment: NSAIDs Kineret Etanercept
  • 23. HIDS Complications Mevalonic aciduria: CNS problems, eye problems, growth/ weight abnormalities
  • 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. TRAPS Treatment: Steroids Etanercept kineret Complication: amyloidosis
  • 26. CAPS FCAS MWS NOMID/CINCA
  • 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. MWS Begins in infancy-adolescent years Rash: hives Joint pain continuous Fatigue Fevers Conjunctivitis
  • 29. MWS Complication: Amyloidosis
  • 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. NOMID/CINCA Complications: Amyloidosis Bony deformities Increased pressures in the skull
  • 32. CAPS treatment Kineret Rilonacept canakinumab