Peripheral nerve involvement in Churg-Strauss syndrome.
Peripheral neuropathy associated with bronchial asthma, multisystem organ dysfunction and idiopathic hypereosinophilia may be found in Churg-Strauss syndrome, hypereosinophilic syndrome and polyarteritis nodosa. Some authors have diagnosed their patients according to the presence in tissue biopsies of the three histological criteria of Churg and Strauss (necrotizing vasculitis, tissue eosinophilic infiltration, extravascular granulomas). We have observed three patients with a common history of a prodromal phase of allergic diseases (bronchial asthma and rhinitis) followed by a vasculitic phase with mononeuritis multiplex, purpura and arthritis, associated with hypereosinophilia of more than 1500 cells/mm3. All responded well to steroid treatment. Sural nerve biopsy revealed true vasculitis in two of these cases and a mild perivascular inflammatory infiltration in the other. On the basis of their characteristic clinical pattern, we think that our cases best fit the diagnosis of Churg-Strauss syndrome even though the typical histological features were not found in the sural nerves examined.
Marazzi
R
R
Department of Neurology, Istituto Neurologico C. Besta, Milan, Italy.
Pareyson
D
D
Boiardi
A
A
Corbo
M
M
Scaioli
V
V
Sghirlanzoni
A
A
D000293:Adolescent; D000328:Adult; D002196:Capillaries / Q000648:ultrastructure; D015267:Churg-Strauss Syndrome / Q000150:complications* / Q000473:pathology; D003593:Cytoplasm / Q000648:ultrastructure; D004730:Endothelium, Vascular / Q000648:ultrastructure; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000209:etiology* / Q000473:pathology; D013497:Sural Nerve / Q000473:pathology