juvenile spondyloarthritis and fever syndromes

Juvenile Spondyloarthritis

Reema Syed,MDAssistant Professor of Internal Medicine and

PediatricsDivision 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

Spondyloarthritis

• Ankylosing spondylitis• Undifferentiated spondyloarthritis• Reactive arthritis• Arthritis associated with IBD• Psoriatic arthritis

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 RheumatologySaint 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