Thrombotic microangiopathy in adult-onset Still's disease: case report and review of the literature

• Published in: Wiener Klinische Wochenschrift 2009-01, Vol.121 (17-18), p.583-588
• Main Authors: Salamon, Lea, Salamon, Tomislav, Morovic-Vergles, Jadranka
• Format: Article
• Language: English
• Subjects: Adult; Anti-Inflammatory Agents - administration & dosage; Case Report; Combined Modality Therapy; Endocrinology; Gastroenterology; Glucocorticoids - administration & dosage; Humans; Internal Medicine; Male; Medicine; Medicine & Public Health; Plasma Exchange; Pneumology/Respiratory System; Still's Disease, Adult-Onset - complications; Still's Disease, Adult-Onset - therapy; Thrombotic Microangiopathies - etiology; Thrombotic Microangiopathies - therapy; Treatment Outcome
• ISSN: 0043-5325; 1613-7671
• DOI: 10.1007/s00508-009-1217-4

Summary Coexistence of thrombotic microangiopathy and adult-onset Still's disease is extremely rare. There is increasing evidence that this association could be more than just coincidental. We report on the case of a 34-year-old male diagnosed with adult-onset Still's disease and successfully treated with intravenous glucocorticoids. Nine months after onset the patient exhibited the presence of asymptomatic thrombocytopenia during treatment with chloroquine. The physical status was unremarkable except for pallor of the skin and mucosa. Laboratory evaluation revealed profound thrombocytopenia and hemolytic anemia. Coombs' tests were negative; renal function tests were all normal. The peripheral blood smear showed frequent schistocytes. Based on the presence of thrombocytopenia and microangiopathic hemolytic anemia, and with the exclusion of other known causes, the patient was diagnosed with thrombotic microangiopathy and successfully treated with plasma exchange and intravenous glucocorticoids. We also review the literature on the association between adult-onset Still's disease and thrombotic microangiopathy; our case is the 15 th report on such an association. The mean age at onset of adult Still's disease in these cases was 31.60 years and the interval between the diagnosis of Still's disease and the onset of thrombotic microangiopathy ranged from 3 days to 17 years, with a female/male ratio of 2 : 1. In more than half the patients thrombotic microangiopathy occurred within the first 6 months after the diagnosis of the Still's disease. Eleven of the 15 (73%) patients were treated with plasmapheresis in addition to glucocorticoid therapy: eight of 11 (73%) had complete remission, the other three had permanent visual impairment and/or digital ischemia. Of the four patients who were not treated with plasmapheresis, two died, one developed end-stage renal disease and one had complete remission. Awareness of the possible development of thrombotic microangiopathy in patients with adult-onset Still's disease is critical, so that treatment can be initiated early and the complications and recurrence of thrombotic microangiopathy prevented. Patients with adult-onset Still's disease should be closely monitored for signs and symptoms of thrombotic microangiopathy during the first six months after diagnosis of the Still's disease.