Meningeal involvement in Wegener's granulomatosis is associated with localized disease.
Clin Exp Rheumatol
; 24(2 Suppl 41): S60-4, 2006.
Article
em En
| MEDLINE
| ID: mdl-16859598
ABSTRACT
Meningeal involvement is a rare occurrence in Wegener's Granulomatosis (WG). A Medline search uncovered only 48 previously reported cases. Here we describe the clinical features of meningeal involvement in WG and to evaluate the association with systemic disease extension. Through a systematic literature review of papers concerning meningeal involvement in WG, we collected and analysed data about sex, age, disease extension, symptoms, cerebrospinal fluid examination, imaging, ANCA and histology about previously reported patients. Headache is almost always the first symptom of meningeal involvement in WG. Later in the course of the disease other abnormalities may develop. Among them cranial nerve palsy, seizures and encephalopathy are the most frequent. Diagnosis is obtained by neuroimaging, which may disclose two distinct patterns of meningeal thickening diffuse or focal. 62.9% of patients tests positive for ANCA. Histology typically shows necrotizing granulomatosis. Meningeal involvement is by far more frequent in the setting of localized WG. Meningitis is a rare complication of WG. It usually develops in patients with localized disease who are more likely to have destructive lesions of the upper airways. It may be recognized by a constellation of clinical and radiological findings and by histological signs of necrotizing granulomatosis, with little or no vasculitis.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Granulomatose com Poliangiite
/
Meningite
/
Meninges
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
/
Risk_factors_studies
/
Systematic_reviews
Limite:
Adult
/
Female
/
Humans
/
Male
/
Middle aged
Idioma:
En
Revista:
Clin Exp Rheumatol
Ano de publicação:
2006
Tipo de documento:
Article
País de afiliação:
Itália