Thanks to Becky Lee yet again for presenting an interesting case of a young woman presenting with acute onset of fever and polyarthritis, found to have a history of similar episodes in the past together with a rash concerning for Still’s disease!
- Still’s disease is a diagnosis of exclusion! Yamaguchi criteria can help with ruling in the diagnosis.
- Still’s remains a multi-systemic disorder of unknown etiology because it’s difficult to diagnose and rare (0.16 cases per 100,000).
- RF and ANA are generally negative but can be positive in <10% of patients with Still’s in low titers.
- ~66% of patients present with sore throat secondary to cricothyroid perichondritis or aseptic nonexudative pharyngitis.
- The disease is often recurrent. Predictors of poor outcome include erosive polyarthritis on presentation and shoulder/hip involvement.
Acute polyarthritis (>5 joints involved):
Remember that for rheumatologic disorders, timing, symmetry, and number of joints involved is crucial to coming up with a differential diagnosis. So for our patient with acute polyarthritis, consider the following:
- Viral: hepatitis, HIV, parvovirus B19
- Spontaneous bacterial endocarditis
- Rheumatoid arthritis
- Reactive arthritis
- Drug reactions
- Auto-inflammatory or disease of the innate immune system (as opposed to autoimmune or diseases of the adaptive immune system):
- Periodic fever syndromes (TRAPS, PFAPA, hyper-IgD syndrome)
- Still’s disease
- Schnitzler’s syndrome – chronic urticaria associated with a monoclonal gammopathy (usually IgM kappa)
- Sweet syndrome – painful skin lesions
- Kikuchi disease – cervical LAD and fever (necrotizing lymphadenitis)
- HLH/Macrophage activation syndrome – leukopenia and thrombocytopenia, elevated triglycerides, low fibrinogen and haptoglobin
- Some clarifications on nomenclature:
- Systemic juvenile idiopathic arthritis (sJIA): first presentation <17 years old, previously referred to as Still’s disease
- Adult onset Still’s disease (AOSD): first presentation > 17 years old
- Epidemiology of AOSD:
- 0.16 cases per 100,000
- No sex predominance (F=M)
- Bimodal age distribution with peak between 15-25 and another 36-46 years of age. New diagnosis in patients >60 have been reported.
- Clinical features ⇒ Yamaguchi criteria (need 5 total with > 2 major)
- Major criteria:
- Daily fevers to 39
- Arthritis >2 weeks
- Non-pruritic salmon-colored macular/maculopapular rash on trunk or extremities (though cases of pruritic rash have also been reported)
- ↑ WBC >10k, >80% neutrophils
- Sore throat
- LAD and/or splenomegaly
- ↑ AST, ALT, or LDH
- Negative ANA/RF
- Major criteria:
- Mild: NSAIDs
- Moderate: NSAIDs + DMARDs
- Severe: NSAIDs + DMARDs (IL1 receptor antagonists like anakinra appear to be more helpful than TNF inhibitors especially in sJIA)
- Overall good prognosis
- Disease can be limited to one episode or recurrent over time
- Poor prognostic indicators:
- Hip and shoulder involvement
- Erosive polyarthritis at initial diagnosis
- Macrophage activation syndrome (ie HLH) can occur in 15% of cases
- Diffuse alveolar hemorrhage
- Pulmonary HTN
- Aseptic meningitis
Great recent review article on Still’s disease (AOSD Review) and this prior post on our blog!