Juvenile Spondyloarthritis and Fever Syndromes
Upcoming SlideShare
Loading in...5
×
 

Juvenile Spondyloarthritis and Fever Syndromes

on

  • 1,206 views

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

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

Statistics

Views

Total Views
1,206
Views on SlideShare
1,206
Embed Views
0

Actions

Likes
0
Downloads
15
Comments
0

0 Embeds 0

No embeds

Accessibility

Upload Details

Uploaded via as Microsoft PowerPoint

Usage Rights

© All Rights Reserved

Report content

Flagged as inappropriate Flag as inappropriate
Flag as inappropriate

Select your reason for flagging this presentation as inappropriate.

Cancel
  • Full Name Full Name Comment goes here.
    Are you sure you want to
    Your message goes here
    Processing…
Post Comment
Edit your comment

Juvenile Spondyloarthritis and Fever Syndromes Juvenile Spondyloarthritis and Fever Syndromes Presentation Transcript

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