Vol.23 No.2

Case Report

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

Authors

Atsushi Masuyama1 , Hitomi Kobayashi1 , Yasuyuki Kobayashi2 , Isamu Yokoe1 , Yusuke Sugimura1 , Keiichiro Maniwa1 , Hiroshi Sato1 , Tsuyoshi Ishida3 , Yuki Hatanaka4

  • Division of Rheumatology, Itabashi Chuo Medical Center, 2-12-7 Azusawa, Itabashiku, Tokyo, 174-0051, Japan
  • Division of Radiology, St. Marianna University School of Medicine, Kawasaki, Japan
  • Division of Pathology, Teikyo University School of Medicine, Tokyo, Japan
  • Division of Neurology, Teikyo University School of Medicine, Tokyo, Japan
Received:

6 October 2011

Accepted:

29 March 2012

Published online:

24 May 2012

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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.

Key words

AOSD - TTP - Cerebral edema - Retinal microangiopathy