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

• Ojima Yoshie

  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

• Nagasawa Tasuku

  Department of Nephrology, Japanese Red Cross Ishinomaki Hospital, Japan

Abstract

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.

Journal

• Internal Medicine

  Internal Medicine 57 (9), 1301-1308, 2018-05-01

  The Japanese Society of Internal Medicine

Keywords

• ANCA-associated vasculitis,
• organ limited vasculitis
• PR3-ANCA

Details

• CRID: 1390282679851030016
• NII Article ID: 130006730798
• DOI: 10.2169/internalmedicine.9848-17
• ISSN: 13497235
• ISSN: 09182918
• PubMed: 29279515
• Web Site: https://www.jstage.jst.go.jp/article/internalmedicine/57/9/57_9848-17/_pdf
• Web Site: https://search.jamas.or.jp/link/ui/2019171078
• Text Lang: en
• Data Source:
  • JaLC
  • Crossref
  • PubMed
  • CiNii Articles
  • KAKEN
• Abstract License Flag: Disallowed