Fulminant course of Wegener's granulomatosis.

Wegener's granulomatosis is a systemic vasculitis associated with necrotizing granulomas. Multiorgan involvement results in a wide range of manifestations. While most patients have an indolent clinical course, a rapidly progressive form is recognized and is the subject of this case report. A 49-year-old white male presented with a 3-week history of dyspnea, cough, and fever. A petechial rash, weight loss, mucosal ulcerations and cyanosis of his fingers were present. His past medical history was noncontributory. The chest radiograph revealed nodular bilateral airspace disease. Bronchoscopy was negative. He had an elevated ESR, rheumatoid factor, and C-ANCA. The initial clinical diagnosis was pneumonia. Within I week of admission, the patient developed clinical signs of myocardial and cerebral infarcts. Additional complications were sepsis, gangrene, and microhematuria. Bronchoalveolar lavage revealed acute inflammation and hemosiderin pigment in macrophages. In an open lung biopsy, H & E stained sections showed parenchymal nodules composed of necrotizing zones in a "geographic" pattern with surrounding granulomatous inflammation and prominent multinucleated giant cells. Small and medium-sized vessels showed necrotizing vasculitis with focal fibrinoid necrosis and mural inflammation. Histochemical stains were negative for acid-fast bacilli, other bacteria and fungi. In Wegener's granulomatosis, early diagnosis is important so that appropriate medical therapy can be instituted. In the context of multiorgan involvement, wedge lung biopsy is the best method of establishing the diagnosis.

Tonialatoya Wallace, MD, Karlene Hewan-Lowe, MD, Charles Gilbert, MD, and James Finley, MD. East Carolina University, Greenville, NC.