Development of MPO-ANCA-related Vasculitis in Systemic Sclerosis: Report of Two Cases and Review of the Literature

Makinodan Eri, Kobayashi Nobuhiko, Hoshino Sachiko, Izumi Atsuko, Yokoi Shoko, Yamamoto Yoshiteru, Asada Hideo, Asai Osamu, Nakatani Kimihiko, Kumamoto Makiko, Suzaki Yasue, Hamada Kaoru, Miyagawa Sachiko

doi:10.14924/dermatol.116.51

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Other Title

MPO-ANCA関連血管炎と全身性強皮症：半月体形成性糸球体腎炎，多発単神経炎を合併した全身性強皮症の2例の検討と文献的考察
MPO ANCA カンレンケッカンエントゼンシンセイキョウヒショウハンゲツタイケイセイセイシキュウタイジンエンタハツタンシンケイエンオガッペイシタゼンシンセイキョウヒショウノ 2レイノケントウトブンケンテキコウサツ

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We report two patients, a 59-year-old female and a 21-year-old female with systemic sclerosis (SSc) who demonstrated myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA). Both patients developed crescentic glomerulonephritis and mononeuritis multiplex, respectively, nine years after the onset of SSc. We diagnosed these patients as having an overlap of SSc and MPO-ANCA-related vasculitis. A combination of oral administration of prednisolone and intravenous cyclophosphamide improved their vasculitic symptoms. We determined the incidence of ANCA-related vasculitis in SSc patients at our hospital. Of the 21 SSc patients examined, only the two patients described here were MPO-ANCA positive. A literature review demonstrated that the features of Japanease SSc patients who produce MPO-ANCA included a high incidence of positive anti-topoisomerase I antibody (71%) and a long lag phase between the onset of SSc and the development of MPO-ANCA (average 10 years, 0.5～30 years). Although the development of MPO-ANCA-related vasculitis is extremely rare in SSc patients, it is clinically of great importance. Acute progressive glomerulonephritis as well as pulmonary hemorrhage caused by ANCA-related vasculitis can determine the prognosis of such patients. If clinically evident vasculitis occurs in an SSc patient, particularly a patient with positive anti-topoisomerase I antibody and a long lag phase from the onset of SSc, prompt diagnosis of ANCA-related vasculitis and the early initiation of immunosuppressive therapy, particularly in the case of life-threatening conditions, can be determinative factors for a good outcome.

Journal

The Japanese Journal of Dermatology

The Japanese Journal of Dermatology 116 (1), 51-60, 2006

Japanese Dermatological Association

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