Zuzana Ríhová, Miroslav Merta, Romana Rysavá, Petr Bezdícek, Vilém Danzig, Karel Gorican, Jindrich Lukás, Pavlína Skalická, Zdenka Vernerová, Vladimír Tesar
CaseRepClinPractRev 2002; 3(1):1-4
Background: We describe a case of Wegener’s Granulomatosis (WG), a disease characterized by necrotizing granulomatous lesions and vasculitis of the upper and lower respiratory tract, necrotizing glomerulonephritis
with crescent formation and systemic vasculitis.
Case report: The patient initially presented with a corneal perforation. In addition to the eye involvement, multiple other organ involvement was found during the course of the disease. The renal biopsy showed a pauciimmune
necrotizing glomerulonephritis with occlusive fibrocellular crescents, the interstitium was focally fibrotic with extensive active inflammation. She had typical upper and lower airway and pulmonary involvement, as well as
the skin vasculitis and arthralgias. Cardiac involvement included an acute myocardial infarction and aortic valve vegetation, most likely caused by WG. Another complication during the initial presentation was a massive
intestinal bleeding. The diagnosis of WG was established by renal, conjunctival and upper airway biopsies and by positive c-ANCA. The clinical and laboratory remission of the disease was achieved by combined immunosuppressive therapy with cyclophosphamide and corticosteroids. Later in the course of the disease the patient developed a subglottic stenosis, most probably due to reparative changes.
Conclusions: In conclusion, this is a case of WG with multiple extrarenal complications, that had a grave clinical course.
Keywords: Wegener’s granulomatosis, ANCA, aortic valvulitis, subglottic stenosis