[Foudroyant course of a therapy resistent Wegener's granulomatosis with negative c-ANCA]

Abstract

Background: Wegener's Granulomatosis is a rare systemic disease characterized by the triad of necrotizing granulomas of blood vessels, upper respiratory tract, and kidneys. Diagnosis depends on clinical symptoms (ACR-criteria), detection of serum circulating antineutrophil cytoplasmic antibodies (c-ANCA) and bioptical histopathology.

Case-report: A 34-year-old patient presented with intraoral ulcerating, necrotizing inflammations of the palate and the gingiva. Chest radiograph (nodules, infiltrates), urin analysis (red blood cell casts) being inconspicuous Wegener's granulomatosis was diagnosed by histopathology of an intraoral incisional biopsy. Conventional therapy with corticosteroids and cyclophosphamide relapsed while the disease was taking a progressive course (pulmonary infiltrates, necrotizing vasculitides all over the common integument). The patient died of cardiac decompensation three months after diagnosis. c-ANCAs were not present at any time of disease.

Conclusions: 1. Symptoms of the head and neck are manifest in up to 95% of Wegener's granulomatosis. So knowledge of the disease is important for dentists and oral and maxillofacial surgeons in spite of its low prevalence. 2. c-ANCA-titers do not reflect disease activity. Negative c-ANCAs do not exclude Wegener's granulomatosis. 3. Wegener's granulomatosis can take a progressive, therapy resistent course.