Case report of atypical Still’s disease: a diagnosis of exclusion

Figures & data

Table 1 Diagnostic criteria of Still’s disease

Five of the following criteria should be met for a diagnosis of ASD. Two criteria must be major

Major criteria

1. Fever of at least 39°C (102.2°F) lasting at least 1 week

2. Arthralgias or arthritis lasting 2 weeks or longer

3. A non-pruritic macular or maculopapular skin rash that is salmon-colored in appearance and usually found over the trunk or extremities during febrile episodes

4. Leukocytosis (10,000/µL or greater), with at least 80% granulocytes

Minor criteria

1. Sore throat

2. Lymphadenopathy

3. Hepatomegaly or splenomegaly

4. Abnormal liver function studies, particularly elevations in AST, ALT and LDH concentrations

5. Negative tests for ANA and RF

Abbreviations: ASD, adult Still’s disease; AST, aspartate transaminase; ALT, alanine transaminase; LDH, lactate dehydrogenase; ANA, anti-nuclear antibody; RF, rheumatoid factor.

Table 2 Classification of Still’s disease and treatment options

Type	Clinical features	Treatment	Dosage
Mild disease	Fever, rash, arthralgia or mild arthritis	Start with NSAIDs alone. 20% respond to this alone. Add low-dose glucocorticoids if symptoms control is not achieved in 10–14 days. Only 20% respond to NSAIDs alone.	Naproxen 500 mg BD, ibuprofen 800 mg TD or QID. Reduce doses as symptoms resolve and continue for 2–3 months. No DMARDs
Moderate disease	High fevers, debilitating joint symptoms, internal organ involvement that is not life threatening or severe	Start with glucocorticoids to control symptoms and inflammatory response. Responds within hours to few days. DMARDs for long-term management or when symptoms recur or cannot be controlled on tapering glucocorticoids. 70% respond to glucocorticoid alone.	Prednisone 0.5 mg to 1 mg/kg. Rapidly taper and maintain at low dose for 2–3 months even when symptoms are controlled. DMARDs anakinra for primarily systemic disease. Methotrexate for primarily articular disease. Taper after patient is stable for 4–6 months starting with glucocorticoid and then DMARD.
Severe disease	Life-threatening organ involvement and/or severe hepatic involvement, cardiac tamponade, DIC	Start with high-dose pulse glucocorticoids and anakinra. Add methotrexate in patients who have ongoing arthritis or develop erosive arthritis. Consider prophylactic antibiotics for pneumocystis pneumonia.	Methylprednisolone 1,000 mg intravascular daily for 3 days. Followed by oral prednisone 60 mg and anakinra 100 mg subcutaneous daily. Taper prednisone as tolerated with the goal of discontinuing it in 2–4 months

Abbreviations: NSAIDs, non-steroidal anti-inflammatory drugs; DMARDs, disease-modifying anti-rheumatic drugs; DIC, disseminated intravascular coagulation.