Meningeal involvement in Wegener's granulomatosis is associated with localized disease

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...

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Bibliographic Details
Published in:Clinical and experimental rheumatology 2006-03, Vol.24 (2), p.S60-S64
Main Authors: DI COMITE, G, BOZZOLO, E. P, PRADERIO, L, TRESOLDI, M, SABBADINI, M. G
Format: Article
Language:English
Subjects:
Adult
Antibodies, Antineutrophil Cytoplasmic - analysis
Biological and medical sciences
Cranial Nerve Diseases - etiology
Cranial Nerve Diseases - pathology
Diseases of the osteoarticular system
Female
Granulomatosis with Polyangiitis - complications
Headache - etiology
Headache - pathology
Humans
Male
Medical sciences
Meninges - chemistry
Meninges - diagnostic imaging
Meninges - pathology
Meningitis - diagnosis
Meningitis - etiology
Meningitis - pathology
Middle Aged
Radiography
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
Seizures - etiology
Seizures - pathology
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Summary: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.
ISSN:0392-856X
1593-098X