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

1. Juvenile Spondyloarthritis
Reema Syed,MD
Assistant 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

7. 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)

8. Juvenile PsA
• Arthritis plus psoriasis
• OR
• Arthritis + 2:
– Dactylitis
– Nail pitting or onycholysis
– Ps in 1st degree relative

9. 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

11. 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

12. 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

13. 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

14. 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

15. Juvenile SpA: treatment
• Sulfasalazine: beneficial for peripheral arthritis
• NSAIDs
• Anti-TNF biologic agents
– Etanercept
– Adalimumab
– Remicade
• Exercise
• PT/OT
• education

16. 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

17. Hereditary Autoinflammatory
diseases: Periodic Fever &
Cryopyrinopathies
Reema Syed,MD
Assistant Professor of Internal Medicine and Pediatrics
Division of Adult and Pediatric Rheumatology
Saint Louis University

18. 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

19. 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)

20. CAPS
• Familial Cold Autoinflammatory Syndrome
(FCAS)
• Muckle Wells Syndrome (MWS)
• Neonatal Onset Multisystem inflammatory
disease (NOMID)/ Chronic Infantile Neurologic
Cutaneous Articular (CINCA) syndrome

21. 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)

22. FMF
• Treatment
– Colchicine
– Kineret (IL-1 receptor antagonist)
– NSAIDs
– Interferon alpha
– Steroids

23. FMF
• Complication
– Amyloidosis

24. 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)

25. HIDS
• Treatment:
– NSAIDs
– Kineret
– Etanercept

26. HIDS
• Complications
– Mevalonic aciduria: CNS problems, eye problems,
growth/ weight abnormalities

27. 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

28. TRAPS
• Treatment:
– Steroids
– Etanercept
– kineret
• Complication:
– amyloidosis

29. CAPS
• FCAS
• MWS
• NOMID/CINCA

30. 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

31. MWS
• Begins in infancy-adolescent years
• Rash: hives
• Joint pain continuous
• Fatigue
• Fevers
• Conjunctivitis

32. MWS
• Complication:
– Amyloidosis

33. 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

34. NOMID/CINCA
• Complications:
– Amyloidosis
– Bony deformities
– Increased pressures in the skull

35. CAPS treatment
• Kineret
• Rilonacept
• canakinumab