Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) restricted to the limbs

Yoshie Ojima, Kinya Sawada, Hiroshi Fujii, Tsuyoshi Shirai, Ayako Saito, Saeko Kagaya, Satoshi Aoki, Yoichi Takeuchi, Tomonori Ishii, Tasuku Nagasawa

Research output: Contribution to journalArticlepeer-review

7 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)1301-1308
Number of pages8
JournalInternal Medicine
Issue number9
Publication statusPublished - 2018


  • ANCA-associated vasculitis
  • Organ limited vasculitis
  • PR3-ANCA

ASJC Scopus subject areas

  • Internal Medicine


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