[Atypical location of Wegener's granulomatosis with breast involvement: case report]

Abstract

Introduction: Wegener's granulomatosis is a disease of unknown etiology associated with the presence of serum antibodies against proteinase 3 in most cases. It is characterized by formation of inflammatory infiltrates presenting as granulomas with fibrinoid necrosis, as well as by ulceration and inflammation of small and medium-sized vessels with the involvement of upper and lower airways and kidneys. The process may also occur in other less typical locations, such as the gastrointestinal tract, heart, and nervous system. There are some reports on the location of lesions in the breast.

Case report: We report a case of a 57-year-old female with Wegener's granulomatosis. In this patient, lesions in the skin, kidneys, upper airways, and lungs were accompanied by a breast tumor revealing the distinctive pattern of an inflammatory granuloma. The disease began in December 2002. The patient experienced painful and swollen joints, fever, mucous-purulent-bloody nasal discharge, and subcutaneous nodules with a tendency to ulceration. Histologically, the nodules had a texture typical for inflammatory granuloma. Renal symptoms included mild proteinuria, abnormalities in the sediment (fresh and leached erythrocytes), and slightly elevated serum creatinine. HRCT of the lungs revealed bilateral pulmonary nodules. The presence of antineutrophil cytoplasmic antibodies in serum with the cytoplasmic fluorescence pattern (c-ANCA/PR-3) was confirmed. The disease progressed with perforation of the nasal septum and extensive destructive changes within the bony structures of the paranasal sinuses. The patient underwent thoracic surgery in 2008 due to an inflammatory tumor in the upper lobe of the right lung. Two years later a tumor in the right breast was detected. Histopathology of the lung and breast tumors showed high similarity of both processes corresponding to lesions typical for Wegener's granulomatosis. The patient was treated with prednisone, methylprednisolone, methotrexate, cyclophosphamide, and azathioprine.

Conclusions: This case provides evidence that Wegener's granulomatosis is a systemic disease with a wide spectrum of organ involvement which should be taken into account during differential diagnosis of breast tumors.