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

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  • Additional Information
    • Source:
      Publisher: Japanese Society of Internal Medicine Country of Publication: Japan NLM ID: 9204241 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1349-7235 (Electronic) Linking ISSN: 09182918 NLM ISO Abbreviation: Intern Med Subsets: MEDLINE
    • Publication Information:
      Original Publication: Tokyo, Japan : Japanese Society of Internal Medicine, [1992-
    • Subject Terms:
      Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/*physiopathology ; Antibodies, Antineutrophil Cytoplasmic/*blood ; Antibodies, Antineutrophil Cytoplasmic/*immunology ; Cyclophosphamide/*therapeutic use ; Microscopic Polyangiitis/*diagnosis ; Muscle Weakness/*drug therapy ; Prednisolone/*therapeutic use; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/immunology ; Asian People ; Biomarkers/blood ; Humans ; Inflammation/blood ; Lower Extremity/physiopathology ; Male ; Middle Aged ; Muscle Weakness/physiopathology ; Myeloblastin/blood ; Myeloblastin/immunology ; Peroxidase/immunology ; Treatment Outcome ; Upper Extremity/physiopathology
    • 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.
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    • Contributed Indexing:
      Keywords: ANCA-associated vasculitis,; PR3-ANCA; organ limited vasculitis
    • Accession Number:
      0 (Antibodies, Antineutrophil Cytoplasmic)
      0 (Biomarkers)
      8N3DW7272P (Cyclophosphamide)
      9PHQ9Y1OLM (Prednisolone)
      EC 1.11.1.7 (Peroxidase)
      EC 3.4.21.76 (Myeloblastin)
    • Publication Date:
      Date Created: 20171228 Date Completed: 20180827 Latest Revision: 20221207
    • Publication Date:
      20221213
    • Accession Number:
      PMC5980815
    • Accession Number:
      10.2169/internalmedicine.9848-17
    • Accession Number:
      29279515