Case Report

Cranial Pachymeningitis: An Unusual Manifestation of Wegener's Granulomatosis

ADEL G. FAM, ELANA LAVINE, LIESLY LEE, BAYARDO PEREZ-ORDONEZ, and MAYANK GOYAL

ABSTRACT.

We describe a patient with limited Wegener's granulomatosis (WG) presenting with saddle-nose deformity, pachymeningitis with prominent visual symptoms due to constriction of ophthalmic arteries at their transdural course, and positive for perinuclear antineutrophil cytoplasmic antibody. Early diagnosis of this rare presentation of WG can facilitate appropriate treatment to avoid irreversible neurologic dysfunction. (J Rheumatol 2003;30:2070-4)

Key Indexing Terms:

WEGENER'S GRANULOMATOSIS PACHYMENINGITIS

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From the Departments of Medicine, Pathology, and Radiology, Divisions of Rheumatology and Neurology, Sunnybrook and Women's College Health Sciences Centre, University Health Network, University of Toronto, Toronto, Ontario, Canada.

A.G. Fam, MD, FRCPC, Professor of Medicine; E. Lavine, Medical Student; L. Lee, MD, FRCPC, Assistant Professor of Medicine (Neurology); B. Perez-Ordonez, MD, FRCPC, Assistant Professor of Pathology; M. Goyal, MD, Neuroradiology Clinical Fellow (currently Associate Professor of Radiology, University of Ottawa).

Address reprint requests to Dr. A.G Fam, Division of Rheumatology, Sunnybrook and Women's College Health Sciences Centre, 2075 Bayview Avenue, M1-401, Toronto, Ontario M4N 3M5, Canada.

Submitted November 8, 2002; revision accepted February 21, 2003.

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