- 著者
- Kenichiro Tokunaga Noboru Hagino
- 出版者
- The Japanese Society of Internal Medicine
- 雑誌
- Internal Medicine (ISSN:09182918)
- 巻号頁・発行日
- vol.56, no.11, pp.1399-1403, 2017-06-01 (Released:2017-06-01)
- 参考文献数
- 15
- 被引用文献数
- 16 37
We performed a retrospective chart review of three patients with hypomyopathic dermatomyositis and rapidly progressive interstitial lung disease. The patients were Japanese women of 71, 69, and 65 years of age. Two patients were anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive and 1 was anti-aminoacyl-tRNA synthetase (anti-ARS) antibody-positive. Their respiratory statuses deteriorated despite the administration of glucocorticoid, calcineurin inhibitors, and intravenous cyclophosphamide therapy. We subsequently administered rituximab. The anti-ARS antibody-positive patient survived, while 2 anti-MDA5 antibody-positive patients died.
- 著者
- Masao Iwagami Kanae Kubo Ryoichi Tanaka Kimito Kawahata Akiko Okamoto Noboru Hagino Kazuhiko Yamamoto
- 出版者
- 一般社団法人 日本内科学会
- 雑誌
- Internal Medicine (ISSN:09182918)
- 巻号頁・発行日
- vol.50, no.20, pp.2413-2416, 2011 (Released:2011-10-15)
- 参考文献数
- 14
- 被引用文献数
- 3 8
We present the first documented case of thrombotic thrombocytopenic purpura (TTP) with severe hypertension complicated by polymyositis and systemic sclerosis sine scleroderma. TTP developed in the progressive phase of visceral fibrosis in the absence of skin thickening. ADAMTS13 activity was not useful for the diagnosis of TTP. Although TTP and scleroderma renal crisis (SRC) share similar findings of thrombotic microangiopathy, severe thrombocytopenia with multiple organ injuries and hemorrhagic manifestations suggested TTP rather than SRC. The patient's condition improved dramatically with plasmapheresis.