Background/Purpose: Cases of Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) during the course of systemic sclerosis (SSc) have recently been reported. Japanese cases of AAV associated with SSc were collected from the literature, and the characteristics of patients in Japan were analyzed.

Methods: A literature review was performed using MEDLINE and the database of the Japan Medical Abstracts Society. The following keywords were used: antineutrophil cytoplasmic antibody; vasculitis; and systemic sclerosis/scleroderma. Case reports, including proceedings and abstracts from Japan that were written in either English or Japanese, were identified. A total of 61 cases were reviewed, including 58 cases from 42 reports (6 in English and 36 in Japanese) and 3 cases from our hospital. The clinical features of SSc-associated AAV were compared with those reported by the AAV survey of the Ministry of Health, Labor and Welfare study group done between 2006 and 2008 in Japan.

Results: In SSc-associated AAV, the average age at onset was 57.0 years, which was 12.4 years earlier than for all AAV patients, and women developed the disease much more frequently than men. In SSc-associated AAV, the male:female ratio was 1:9, whereas the ratio was 1:1 in all AAV. In SSc, the proportions of the diffuse and the limited types were equal; anti-Scl-70 antibody was positive in 35 cases, and anti-centromere antibody was positive in 6 cases. The mean duration between diagnosis of SSc and AAV was 10.3 years (range, 1–41 years). In AAV, alveolar hemorrhage developed in 24%, and rapid progressive glomerulonephritis developed in 81%; both were more frequent than in all AAV as reported by the survey (11% and 63%, respectively). On the other hand, neurological manifestations were less frequent in SSc-associated AAV (24% versus 45%). No cases were positive for anti-PR3 antibody. Twelve cases died, and the most frequent cause of death was infection (7 cases).

Conclusion: SSc-associated AAV in Japan has several characteristics, including younger onset, female predominance, frequent pulmonary and renal manifestations, and long duration from SSc to AAV. It should be noted that this association is not extremely rare, and increased awareness may lead to earlier diagnosis and better prognoses.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/antineutrophil-cytoplasmic-antibody-associated-vasculitis-associated-with-systemic-sclerosis-in-japan-a-review-of-the-literature/