Severe ARDS and secondary haemophagocytic lymphohistiocytosis complicating a flare of adult-onset Still's disease: a case-based review.
Lohith Karigowda, Vanessa Wong, Anna Tran et al.
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Case in brief
A 42-year-old woman with adult-onset Still’s disease (AOSD) developed a flare after a five-week interruption of conventional synthetic disease-modifying therapy around surgery for a perianal abscess with fistulous disease. She presented with severe hypoxaemic respiratory failure, requiring intubation and later prone positioning for acute respiratory distress syndrome (ARDS). She subsequently developed macrophage activation syndrome/secondary haemophagocytic lymphohistiocytosis, supported by hyperferritinaemia, hypertriglyceridaemia, fever and marrow-confirmed haemophagocytosis; Epstein–Barr and cytomegalovirus reactivation also complicated the course. After sequential multi-target immunomodulation with antimicrobial cover, she survived, left intensive care on day 34 and was discharged on day 73.
What made this case unusual
The pulmonary manifestation was fulminant ARDS rather than the more indolent infiltrative lung disease typically described in AOSD. The authors note that concomitant infection and viral reactivation prevent attributing the illness to AOSD alone.
Diagnostic / clinical pearl
In a patient with AOSD and severe respiratory failure, consider that pulmonary disease and MAS-HLH may occur together; assess for MAS-HLH while also evaluating for infection and viral reactivation.
Why it matters
Survival followed multidisciplinary immunomodulation alongside antimicrobial cover, but the sequential use of several agents means this case cannot establish the efficacy of any individual treatment or combination.