Adult-Onset Still’s Disease in a Patient with Macrophage Activation Syndrome and Pre-Disseminated Intravascular Coagulation: A Case Report and Literature Review

Jingjuan Xu,* Jing Yang,* Yue Hu, Zhiming Zhao, Hui Cai, Qifeng Yao, Wei Shang Department of Traditional Chinese Medicine, Jinling Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, Jiangsu, 210002, People’s Republic of China*These authors contributed equally to this workCorrespondence: Qifeng Yao, Department of Traditional Chinese Medicine, Jinling Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, Jiangsu, 210002, People’s Republic of China, Email [email protected] Wei Shang, Department of Traditional Chinese Medicine, Jinling Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, Jiangsu, 210002, People’s Republic of China, Email [email protected]: 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.Keywords: adult-onset Still’s disease, macrophage activation syndrome, pre-disseminated intravascular coagulation, glucocorticoids, tofacitinib