title: 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.
authors.affiliation: Division of Rheumatology, Itabashi Chuo Medical Center, 2-12-7 Azusawa, Itabashiku, Tokyo 174-0051, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Masuyama
authors.forename: Hitomi
authors.initials: H
authors.lastname: Kobayashi
authors.forename: Yasuyuki
authors.initials: Y
authors.lastname: Kobayashi
authors.forename: Isamu
authors.initials: I
authors.lastname: Yokoe
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Sugimura
authors.forename: Keiichiro
authors.initials: K
authors.lastname: Maniwa
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Sato
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Ishida
authors.forename: Yuki
authors.initials: Y
authors.lastname: Hatanaka
mesh_terms: D000328:Adult; D001929:Brain Edema / Q000150:complications* / Q000473:pathology; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications* / Q000473:pathology; D012164:Retinal Diseases / Q000150:complications* / Q000473:pathology; D016706:Still's Disease, Adult-Onset / Q000150:complications* / Q000473:pathology; D057049:Thrombotic Microangiopathies / Q000150:complications* / Q000473:pathology