Take-home pearl: when a patient presents with arthralgias and a rash, we often think about infection (esp viral), autoimmune, malignancy (and meds). The time course can help! Symptoms for 1-2 weeks may point towards viral, whereas longer may suggest autoimmune.
Approach to joint pain: helpful to narrow your ddx! Check out these old PEARLS for more (incidentally, this link shares pearls from a separate Still’s case from November!)!
- arthritis (is there inflammation?) vs arthralgias
- time course (acute à think about infectious and autoimmune causes)
- # of joints involved (mono, oligo, vs poly) and distribution/symmetry
Pearls on adult onset Still’s disease:
- Often diagnosed months after presentation since it is a diagnosis of exclusion
- Thanks to Vivek for sharing a pearl from one of the rheum attendings: patients with Still’s often spike fevers in the late afternoon. Per her, this is highly specific!
- The characteristic rash in Still’s is characteristically nonpruritic, evanescent (correlates with time of fever), and salmon-colored! Distribution is often on the chest.
- However, there are case reports of urticarial rashes as the presenting manifestation of Still’s! (PMID 11055830, PMID 17241583)
- Whenever Still’s is on your ddx, per our derm and rheum colleagues, you should always also entertain Schnitzler’s syndrome!
- Schnitzler’s – recurrent urticarial rash (neutrophil predominant), monoclonal gammopathy, with signs of systemic inflammation. Like patients with Still’s, they are often patients with FUOs and have minor criteria very similar to those patients with Still’s. Treatment includes colchicine, NSAIDs, and if severe inflammation, DMARDS (like anakinra); steroids are not A review article is attached!
- Still’s – as above, usually is Treatment is ASA or NSAIDs, steroids (sometimes pulsed) (95% effecitive), and DMARDs
- While not part of the diagnostic criteria, high ferritin is characteristic, and the higher the ferritin, the greater the disease activity!
- Yamaguchi criteria is the most widely used for diagnosis – patients must fulfill 5 of the below criteria, 2 of which should be major (not to be memorized!)
- Prognosis: predictors of unfavorable outcomes/chronic disease: erosive polyarthritis at initial presentation, involvement of shoulders or hips. Systemic symptoms are generally not predictive of poorer outcomes!