Adult-Onset Still's Disease in a Patient with Macrophage Activation Syndrome and Pre-Disseminated Intravascular Coagulation: A Case Report and Literature Review
Abstract
Background: Adult-onset Still's disease (AOSD) is a rare autoinflammatory condition characterized by high spiking fevers, arthralgia, typical rash, and systemic inflammation. Life-threatening complications, such as macrophage activation syndrome (MAS) and disseminated intravascular coagulation (DIC), may arise. Prompt recognition and intensive management are crucial for patient survival. This report presents a case of AOSD complicated by MAS and pre-DIC, accompanied by a review of the relevant literature.
Case presentation: A 22-year-old male presented with recurrent high-grade fever, generalized red rash, lymphadenopathy, splenomegaly, and knee arthralgia. He met the Yamaguchi criteria for AOSD and the Ravelli criteria for MAS, and was diagnosed with pre-DIC using the Chinese Diagnostic Scoring System for DIC (CDSS). Following diagnosis, the patient received a comprehensive medical regimen that included glucocorticoids, the JAK inhibitor tofacitinib, infusion of fresh frozen plasma, and supportive care, resulting in clinical improvement. At the 8-month follow-up evaluation, the patient's condition remained stable without any signs of progression.
Conclusion: This case underscores the critical importance of early identification of MAS and pre-DIC in AOSD, and demonstrates that aggressive immunosuppressive and supportive therapy, implemented within a multidisciplinary framework, is essential to prevent progression to overt DIC and multi-organ failure.
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Evidence Hub reads this as a case report, done in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health.
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