Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis (AAV) Restricted to the Limbs

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Author(s)

    • Ojima Yoshie
    • Department of Nephrology, Japanese Red Cross Ishinomaki Hospital, Japan
    • Nagasawa Tasuku
    • Department of Nephrology, Japanese Red Cross Ishinomaki Hospital, Japan
    • Sawada Kinya
    • Department of Radiology, Takeda General Hospital, Japan
    • Fujii Hiroshi
    • Department of Hematology and Rheumatology, Tohoku University School of Medicine, Japan
    • Shirai Tsuyoshi
    • Department of Hematology and Rheumatology, Tohoku University School of Medicine, Japan
    • Saito Ayako
    • Department of Nephrology, Hypertension and Endocrinology, Tohoku University School of Medicine, Japan
    • Kagaya Saeko
    • Department of Nephrology, Japanese Red Cross Ishinomaki Hospital, Japan
    • Aoki Satoshi
    • Department of Nephrology, Japanese Red Cross Ishinomaki Hospital, Japan
    • Takeuchi Yoichi
    • Department of Nephrology, Japanese Red Cross Ishinomaki Hospital, Japan
    • Ishii Tomonori
    • Clinical Research, Innovation and Education Center, Tohoku University Hospital, Japan

Abstract

<p>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 creatine kinase (CK) level increased to 29,998 U/L. Methylprednisolone (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. </p>

Journal

  • Internal Medicine

    Internal Medicine 57(9), 1301-1308, 2018

    The Japanese Society of Internal Medicine

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