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Diagnostic role of peripheral nerve biopsy in Churg‐Strauss Syndrome

Sonia Narendra,Christine Fuller-2008-03-01-The FASEB Journal
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TL;DRAbstract

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 infiltr

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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 infiltr

Keywords

Mononeuritis MultiplexMedicineNecrotizing VasculitisEosinophiliaVasculitisPathologyPeripheral neuropathyNerve biopsy

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