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Diagnostic role of peripheral nerve biopsy in Churg‐Strauss Syndrome
Author(s) -
Narendra Sonia,
Fuller Christine
Publication year - 2008
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.22.1_supplement.708.29
Subject(s) - mononeuritis multiplex , medicine , necrotizing vasculitis , eosinophilia , vasculitis , pathology , peripheral neuropathy , nerve biopsy , biopsy , eosinophil , panca , peripheral , asthma , disease , anti neutrophil cytoplasmic antibody , diabetes mellitus , endocrinology
Churg‐Strauss syndrome (CSS) is an uncommon yet distinctive form of systemic necrotizing vasculitis. Though the diagnosis is often made on clinically grounds by the presence of adult‐onset asthma, peripheral eosinophilia, and vasculitis‐associated symptoms, tissue confirmation may be necessary in some cases. Herein we report a case of a 52 year‐old woman recently diagnosed with asthma who presented with acute worsening of pulmonary symptoms and new onset of mononeuritis multiplex. Her weakness and numbness began asymmetrically in her arms, progressing to involve both legs over one week. Laboratory findings included peripheral eosinophilia, elevated IgE and rheumatoid factor, and positive pANCA. Radioimaging studies revealed bilateral pulmonary infiltrates and mediastinal adenopathy. Nerve conduction studies documented diminished amplitude and conduction block in multiple tested nerves. Radial nerve biopsy revealed active axonal degeneration, vascular and perivascular eosinophil infiltrates, and rare granulomas. A diagnosis of CSS was established based on American College of Rheumatology criteria. She later developed ischemic bowel secondary to necrotizing vasculitis (established microscopically in resection specimen). In cases of CSS where peripheral nerve damage dominates the clinical picture, peripheral nerve biopsy may be critical in establishing an early diagnosis.