A case of adult-onset Still’s disease complicated by thrombotic thrombocytopenic purpura with retinal microangiopathy and rapidly fatal cerebral edema

Abstract

We present a patient who had adult-onset Still's disease (AOSD) complicated by thrombotic thrombocytopenic purpura (TTP) that resulted in retinal microangiopathy and rapidly fatal cerebral edema. The patient was a 37-year-old male who developed fever, eruption, arthritis and hepatic dysfunction, that, based on close examination, was diagnosed as AOSD. Despite treatment with corticosteroids, the patient developed acute visual field defect, neurological deterioration including convulsions and impaired consciousness, as well as acute renal failure that ultimately resulted in death. Pathological examination of autopsy specimens revealed multiple fibrin thrombi disseminated in small vessels of the brain and kidney, which was consistent with TTP, along with marked cerebral edema. Although TTP has rarely been reported in association with AOSD, awareness of the possible coexistence of these two diseases is important for diagnosis and treatment.

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

“…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. Our patient's clinical presentation was similar to what had been previously reported.…”

Mechanisms, biomarkers and targets for adult-onset Still’s disease

“…TMA treatment is complex and mandatorily includes the following 119 : supportive care in an ICU with plasma exchange being central 133 and high-dose steroids intravenously and then orally. Other immunomodulatory agents proposed for refractory TMA cases include anakinra 130 , tocilizumab 135 , intravenous immunoglobulins, cyclophosphamide, azathioprine, ciclosporin A and rituximab 119,130 . Despite this treatment, mortality associated with TMA remains high (up to 20%) 119 .…”

Adult-onset Still's Disease with Acute Kidney Injury Requiring Hemodialysis: A Case Report and Literature Review

“…To analyze the clinical characteristics of AOSD cases with severe renal involvement, such as our case, we collected and reviewed cases of AOSD presenting with severe AKI requiring HD (Table 2). To our knowledge, only 12 cases of AOSD with severe AKI requiring HD and with a confirmed cause by renal biopsy have been reported (7,8,(13)(14)(15)(16)(17)(18)(19)(20)(21). Renal impairment was caused by renal amyloidosis in 4 cases, TMA in 6 cases, and CG in 2 cases.…”