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

Abstract

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.

Emergency Mechanical Thrombectomy for Acute Middle Cerebral Artery Occlusion Accompanied by Adult-Onset Still’s Disease

“…Association has also been reported between AOSD and thrombotic microangiopathy. In more than half the patients, thrombotic microangiopathy occurred within the first six months after the diagnosis of AOSD [ 5 ]. As described above, thrombocytosis or vasculitis is a major cause of cerebral infarction in AOSD patients.…”

Thrombotic microangiopathy in a patient with adult‐onset Still's disease

“…By searching PubMed, we identified 20 cases of TMA associated with AOSD. The most recent literature review was published in 2009 and included a total of 15 cases . An additional five cases have been published since this review 8‐12.…”

Adult‐onset Still's disease with concurrent thrombotic microangiopathy: Observations from pooled analysis for an uncommon finding

“…2 The primary clinical features of AOSD include daily fevers, arthritis, leukocytosis (>10 000 cells/μL with neutrophils >80%), and a transient salmon-pink maculopapular rash. While not comprehensive, hematological manifestations in AOSD include symmetrical lymphadenopathy, 3 splenomegaly, 3 macrophage activation syndrome, 3 thrombotic microangiopathy (TMA), 4 and disseminated intravascular coagulation. 5 The recognition and diagnosis of AOSD are critical due to the potential for severe complications, making prompt and appropriate treatment essential.…”