Transcript 5.22.09 Paulsen

Adult Still’s Disease
Grant Paulsen
AM Report
May 22, 2009
What is it?
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Inflammatory disease with symptoms of
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Still’s Disease first described in 1896; now called
systemic onset juvenile inflammatory arthritis
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Daily fevers
Arthritis
Rash (evanescent)
Formerly know as JRA (juvenile rheumatoid arthritis)
‘Adult’ Still’s describes adult patients with similar
features to the childhood disease, but don’t meet
RA criteria for adult RA.
Basics
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Etiology
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Basically no one knows. Possibly due to viral or
bacterial trigger. No confirmed genetic link,
familial cases are rare.
Epidemiology
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Approx 0.16 cases per 100,000
Equal male:female ratio
Bimodal peaks of incidence
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Age 15-25 & age 36-46
Diagnostic Criteria
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In the past there have been up to 7 (yep, seven) sets of
diagnostic criteria.
Yamaguchi Criteria (need 5, with at least 2 major)
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Major
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Fever - at least 39.0 for at least 1 week
Arthralgias/Arthritis - 2 weeks, or more
Rash – salmon-colored, nonpruritic or maculopap, often during
febrile episodes.
Minor
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Sore Throat
LAD
Hepatomegaly or Splenomegaly
Abnormal LFT’s – Commonly, elevated AST, ALT, LDH
Negative ANA, and RF
Details
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Fever
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Daily, often with dramatic temp swings
Rash
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Often involves trunk and extremities, but can go to face, palms, soles
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Koebner Phenomenon – Rash after stroking the skin
Frequently mis-diagnosed as drug rash
Path – Perivascular inflammation (non-specific)
Arthralgia/Arthritis
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Often start as mild monoarticular to oligoarticular
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Progress to severe, destructive polyarthritis over months
 May lead to fusion of wrist joints
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Most frequent joints – knees, wrists, ankles, elbows, PIP, shoulders
Pharyngitis
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Severe, non-suppurative
Clinical Presentation
cont’d
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Liver Disease
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LAD & Splenomegaly
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Elevated AST, ALT
Often cervical, and often takes people down the lymphoma path
Cardiopulmonary
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Pericarditis
Pleural effusions
Pulmonary infiltrates – transient, non-infectious.
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Heme
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Severe ILD – rare, but has been reported
Reactive Hemophagocytic Syndrome (Macrophage activation)
DIC
MAHA – including TTP/HUS
Alopecia
Lab Abnormalities
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Lots of ‘characteristic’ abnormalities, but none are
truly specific
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Elevated CRP, ESR
Leukocytosis – often >15,000
Anemia – normochromic, normocytic
Thrombocytosis – reactive
Elevated AST, ALT – mild all the way to fulminant
necrosis
Elevated Ferritin – often >3000, which is rare in the other
Rheum diseases
Bone Marrow – hyperplasia of granulocyte precursors,
hypercellularity, histiocytosis, hemophagocytosis
Radiographic
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X-Rays
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Wrist films – narrowing of carpo-metacarpal and
intercarpal joint spaces, leading to ankylosis
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Late finding
Stop. Too many details
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What to remember
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Think about Still’s after you’ve chased the
common sites of infection/fever
Start thinking about it when you see
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Daily fevers associated with rash/joint pain
High CRP, ESR; but negative ANA, RF
Very high ferritin levels
Sore Throat – non-exudative
Course
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Monophasic
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Lasts <1 year and resolves completely
Intermittent
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Have flares with complete remission between episodes
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Flares are often less severe
Chronic
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Persistently active, often going to destructive arthritis
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May ultimately need total joint arthroplasty
Treatment
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NSAIDs first
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Use standing doses (Ibuprofen 800 QID)
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Steroids next
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If present with debilitating symptoms, internal organ
involvement, persistent high fever, start with steroids.
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Monitor for reactive hemophagocytic syndrome
Pred 0.5-1.0mg/kg/day
Methylpred pulse if severe/life-threatening symptoms
Biologics (newer, but not yet standard of care)
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TNF-alpha inhibitors – infliximab, etanercept, adlimumab
Rituximab – Often used after TNFa’s fail
Anakinra (IL-1 antagonist) – currently last line, due to insufficient
data
Treatment
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DMARDs
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Methotrexate
Cyclosporine
Hydroxychloroquine
Cyclophosphamide
Azathioprine
IVIG
References
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www.utdol.com.
Arlet JB. Huong DL. Et al. Reactive haemophagocytic
syndrome in adult-onset Still’s disease: a report of six
patients and a review of the literature. Ann Rheum Dis
2006;65;1956-1601.
Mert A. Ozaras R. et al. Fever of unknown origin: a review of
20 patients with Adult-onset Still’s disease. Clin Rheumatol
2003;22:89-93.
Zeng T. Zou YQ. et al. Clinical features and prognosis of
Adult-onset Still’s disease: 61 cases from China. J Rheum
2009;36:1-6.