Neuro-muscular biopsy in Churg-Strauss syndrome: 24 cases.
Journal of Neuropathology and Experimental Neurology. 2006-02-01; 65(2): 187-192
DOI: 10.1097/01.jnen.0000200151.60142.25
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1. J Neuropathol Exp Neurol. 2006 Feb;65(2):187-92. doi:
10.1097/01.jnen.0000200151.60142.25.
Neuro-muscular biopsy in Churg-Strauss syndrome: 24 cases.
Vital A(1), Vital C, Viallard JF, Ragnaud JM, Canron MH, Lagueny A.
Author information:
(1)Department of Neuropathology BP 42, Victor Segalen-Bordeaux 2 University, 146
rue Léo-Saignat, 33076 Bordeaux cedex, France.
Churg-Strauss syndrome (CSS) is a distinctive clinical entity in which systemic
vasculitis, associated with eosinophilia, occurs almost exclusively in
individuals with adult-onset asthma. The major complications of the condition
result from damage to the lungs, heart, and peripheral nerves. Necrotizing
vasculitis with eosinophils in the cellular infiltrate, vascular or perivascular
infiltration by eosinophils in absence of vessel wall necrosis, extra-vascular
eosinophil infiltrates, and vascular or extra-vascular granuloma are
histopathological features supportive of CSS. As the peripheral nerve disease
often dominates the clinical picture, the peripheral nerve biopsy may be
decisive in establishing the diagnosis. In this retrospective study of
neuro-muscular biopsies in 24 CSS cases, the authors give an extensive
description of neuropathological lesions associated with this disorder. Fifteen
patients (62.5%) exhibited eosinophils either in extra-vascular infiltrates or
in vessel walls, and 6 of them (25%) had an associated necrotizing vasculitis.
Granulomas were found in only 3 cases (12.5%). The clinical diagnosis of CSS was
supported in 15 out of the 24 patients (62.5%), in the nerve in 2 cases (8.3%),
in the muscle in 8 cases (33.3%), and in both nerve and muscle in 5 others
(20.8%).
DOI: 10.1097/01.jnen.0000200151.60142.25
PMID: 16462209 [Indexed for MEDLINE]