Abstract

Wegener’s disease, currently called granulomatosis with polyangiitis, is a systemic necrotizing vasculitis of small vessels. It is typically associated with nodular opacity type pulmonary lesions of varying size developing towards excavation. Pulmonary fibrosis is more rarely observed. We report a case initially believed to be a Sjögren’s syndrome in a 32-year old female Senegalese patient. The diagnosis of Wegener’s granulomatosis was given on the basis of pulmonary fibrosis, oral ulcers, and the positivity of anti-PR3 type ANCA. The response to treatment remains favorable to this day, despite the occurrence of a secondary intercostal herpes zoster upon the use of cyclophosphamide and corticosteroids. The treatment of Sjögren’s syndrome, for which the criteria were not met as it happens, may have bastardized the clinical expression of Wegener’s disease.

Keywords: Sjögren’s syndrome, wegener’s disease, granulomatosis, pulmonary fibrosis