Adult-onset Still’s disease with disseminated intravascular coagulation and hemophagocytic syndrome: a case report

BACKGROUND: Adult-onset Still’s disease is a rare inflammatory condition of unknown origin characterized by high spiking fever, arthralgia, arthritis, myalgia, salmon-colored evanescent rash, and hepatosplenomegaly. The diagnosis of adult-onset Still’s disease requires the exclusion of other possible disorders because it lacks specific clinical and histopathological findings. Adult-onset Still’s disease rarely become fatal due to visceral involvements such as disseminated intravascular coagulation. CASE PRESENTATION: A 22-year-old Chinese female presented to our medical center with high spiking fever for one week, myalgia for two weeks, and arthralgia and pink maculopapular rash for four weeks. She developed disseminated intravascular coagulation on the fourth day after admission. There was no other explanation for the fever and rash, including infection, malignancy, and collagenosis. Together, the high spiking fever, salmon-colored rash, splenomegaly, and excess hepatic enzyme, indicated adult-onset Still’s disease based on the Yamaguchi criteria. Therefore, prednisolone therapy was initiated. The combination of nafamostat mesilate and prednisolone therapies caused a rapid reduction in the fever and rash. The inflammatory markers decreased immediately, and disseminated intravascular coagulation improved. Her symptoms resolved with low-dose prednisolone treatment, and she was monitored thereafter at our outpatient clinic. CONCLUSION: The previous use of nonsteroidal anti-inflammatory drugs could have caused disseminated intravascular coagulation in this patient with adult-onset Still’s disease. We propose that physicians should consider the possibility of disseminated intravascular coagulation as a complication during the course of adult-onset Still’s disease and suggest that prednisolone therapy should be initiated in the early stages of adult-onset Still’s disease.