The anti-neutrophil cytoplasmic antibody-associated vasculitides include granulomatosis with polyangiitis (Wegener's granulomatosis) and microscopic polyangiitis. The introduction of therapy with cytotoxic agents such as cyclophosphamide transformed these diseases from fatal diagnoses to chronic conditions characterized by cycles of relapse and remission. Modern treatment strategies have focuse...

We report a case of bilateral acute angle-closure glaucoma in a patient with undiagnosed granulomatosis with polyangiitis (Wegener's). A 59-year-old man presented with a severe headache, ocular pain, blurred vision, shortness of breath, and mild fever. Clinical examination revealed conjunctival chemosis, corneal edema, and shallow anterior chambers. Closed angles were observed bilaterally on go...

A 26 year old man with subacute hoarseness and stridor was shown to have Wegener's granulomatosis isolated to the trachea and larynx. Although isolated laryngeal Wegener's is unusual, a review of the literature suggests that early treatment with cyclophosphamide is warranted.

Wegener's granulomatosis (WG) is a rare, systemic vasculitis involving multiple organs. The clinical presentation is highly diverse, and there is considerable risk of mortality if diagnosis and treatment are delayed. We present a case illustrating that patients with WG may initially present with localized cutaneous symptoms and signs.

1. Cotch MF, Hoffman GS, Yerg DE, Kaufman GI, Targonski P, Kaslow RA. The epidemiology of Wegener's granulomatosis. Estimates of the five-year period prevalence, annual mortality, and geographic disease distribution from population-based data sources. Arthritis Rheum. 1996;39(1):87-92. 2. McDonald TJ, Deremee RA. Wegener's granulomatosis. Laryngoscope. 1983;93(2):220-31. 3. Fauci AS, Haynes BF,...

Introduction Granulomatosis with polyangiitis (GPA) is a necrotising granulomatous vasculitis affecting the small and medium blood vessels in particular of airways and kidneys. The incidence of GPA in Europe is 25-150 per 1 million per year. This disease typically occurs in the 4th or 5th decades of life and in children it usually cause diagnostic and therapeutic difficulties. Subglottic stenos...

Eight patients who had limited forms of Wegener's granulomatosis are described, with details of their pathology. Ocular pathological data were available for 2 of them. The condition carries a serious ocular risk; useful vision was lost in 6 out of 16 eyes (37%). An indolent but slowly progressive marginal keratitis and scleritis was a prominent feature in 4 patients and was helpful in suggestin...

Wegener 's granulomatos is , a d isease of unknown et io logy, is character ized by a granulomatous vascul i t is of the upper and lower r e s p i r a t o r y t r a c t s . Va r y i n g d e g r e e s o f s y s t e m i c v a s c u l i t i s o f s m a l l arter ies and veins may also be seen <1>. Renal disease in the form of f o c a l o r d i f f u s e n e c r o t i z i n g g l o m e r u l i t i ...

Granulomatosis with polyangiitis (GPA; Wegener's granulomatosis) is a systemic necrotizing vasculitis of unknown etiology that commonly involves the upper airways, lungs, and kidneys. Cardiac involvement with an intracardiac mass is an exceedingly rare manifestation of this disease, especially in the pediatric population where, to our knowledge, only one article exists to date that has describe...