Shock in Adult-Onset Still's Disease Complicated by Macrophage Activation Syndrome: Clinical Characteristics and Prognosis of 14 Patients.

Background: Shock is a rare life-threatening complication in patients with adult-onset Still's disease (AOSD) complicated by macrophage activation syndrome (MAS). This study aims to summarize the clinical characteristics and prognosis of this condition. Methods: We performed a retrospective study of 14 patients hospitalized from January 2017 to December 2023 who experienced shock secondary to AOSD complicated by MAS. Results: Among the 14 patients, 11 presented with a progressive rash, and 10 exhibited a remittent fever prior to shock onset. Compared to levels at admission, serum ferritin (SF), lactate dehydrogenase (LDH), and procalcitonin levels were significantly elevated, while serum albumin (ALB) levels were significantly decreased, both at the time of MAS diagnosis and at the onset of shock. Shock resolved in nine patients, and five died. All the improved patients were treated with high-dose glucocorticoids (equivalent prednisolone dose ≥ 1 mg/kg/day) prior to MAS diagnosis, and received glucocorticoid pulse therapy. Among them, five (55.6%) received combination therapy with low-dose etoposide and cyclosporine, one (11.1%) received etoposide alone, one (11.1%) received a combination of cyclosporine and tocilizumab, and two (22.2%) receive no additional immunosuppressive therapy. Of the five patients who died, four (80%) received glucocorticoids therapy (two before MAS diagnosis), and only two received a combination of cyclosporine. None of the deceased patients received tocilizumab or etoposide. Compared to patients who died, the patients who improved had significantly higher rates of early glucocorticoid therapy (100% vs. 40%, p = 0.027) and etoposide use (66.7% vs. 0%, p = 0.028). Conclusion: Shock is a life-threatening condition secondary to AOSD with MAS. The early combined use of high-dose glucocorticoids and etoposide may be key to improving outcomes in this critical condition.