The course of granulomatosis with polyangiitis (Wegener’s granulomatosis) with late ocular and orbital manifestations (clinical observation)

Abstract

Granulomatosis with polyangiitis (GPA) is a granulomatous necrotizing vasculitis associated with antineutrophil cytoplasmic antibodies. The clinical picture of GPA is characterized by a wide range of manifestations, including nonspecific symptoms, signs of damage to the upper and lower respiratory tract and glomerulonephritis. The article presents the observation of a patient for 12.5 years, who at the beginning of the disease, along with complaints of malaise, weakness, decreased appetite and weight loss, had a hemorrhagic rash, proteinuria, erythrocyturia, decreased glomerular filtration rate and increased creatinine and urea levels. After a positive result for antineutrophil cytoplasmic antibodies and the results of nephrobiopsy with evidences of glomerulonephritis with the development of glomerulosclerosis and fibrosis of the crescents (pauci-immune variant) with a moderately pronounced tubulointerstitial component, the patient was diagnosed with GPA and immunosuppressive therapy was initiated. Almost 8 years after the manifestation of GPA during continuous pathogenetic therapy, the patient complained of swelling of the left eye and its increase in volume, diplopia. Computed tomography of the head revealed a mass of the left orbit. Morphological examination of the excised mass revealed its inflammatory origin as consequence of vasculitis. The special aspect of the presented case is that ocular and orbital pathologies of GPA developed in the patient 8 years after the initial manifestation of the disease against the background of ongoing immunosuppressive therapy in conditions of laboratory remission of GPA.