Granulomatosis with polyangiitis – diagnostic difficulties?

Granulomatosis with polyangiitis (GPA) is characterized by necrotic vasculitis of the medium and small vessels, granuloma formation and presence of antineutrophil cytoplasmic antibodies against proteinase-3 in serum. The upper respiratory tract, lower respiratory tract and kidneys are usually involved in the disease. Atypical clinical presentation may generate problems. In this report we present a case in which the diagnosis of GPA was possible, but was verified during in further clinical observation. We present the medical history of a 70-year-old woman with ocular symptoms, nasal discharge, progressive hearing impairment, craniofacial inflammatory infiltrates, lymphadenopathy, and microscopic haematuria in laboratory tests. Histopathology did not confirm GPA. cANCA tests were also negative. Finally, after mediastinoscopy and histopathological evaluation of the lymph nodes, the diagnosis of B-cell lymphoma was established.