Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
Anti-Neutrophil Cytoplasmic Antibody-associated Vasculitis (AAV) Restricted to the Limbs: A Case Report
Yoshie OjimaKinya SawadaHiroshi FujiiTsuyoshi ShiraiAyako SaitoSaeko KagayaSatoshi AokiYoichi TakeuchiTomonori IshiiTasuku Nagasawa
ジャーナル オープンアクセス 早期公開

論文ID: 9848-17


A previously healthy 58-year-old man was admitted for muscle pain and weakness (manual muscle testing [MMT] of 4/4 for upper and lower limbs). We detected elevated levels of inflammatory makers and PR3-anti-neutrophil cytoplasmic antibody (ANCA). Subsequently, the muscle weakness rapidly progressed to an MMT of 2 for all limbs. Magnetic resonance imaging indicated muscle edema, and the CK level increased to 29,998 U/L. mPSL and cyclophosphamide pulse therapy improved the patient symptoms. MMT recovered to 4 for all limbs. A muscle biopsy showed degenerated muscle fibers surrounded by neutrophil-predominant infiltration. In addition, lamina elastic breakdown and fibrinoid necrosis of arterioles were observed. A final diagnosis of microscopic polyangiitis (MPA) limited to the muscles was made.

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