Abstract:
Abstract. Granulomatosis with polyangiitis (GPA)(Wegener's) is a disease from the group of systemic vasculitis, which is characterized by necrotizing granulomatous inflammation and necrotizing vasculitis of small vessels with a predominant lesion of the upper respiratory tract, lungs and kidneys.
The study involved 60 patients (29 men and 31 women) aged 18 to 80 years with an established diagnosis of GPA. The average age of the examined patients was 48.9 ± 15.6 years. The diagnosis of GPA (n = 60) was established in accordance with the nomenclature adopted in 2012 at the conference in Chapel Hill (USA), as well as on the basis of the presence of at least 2 of 4 criteria of the American College of Rheumatology (ACR), 1990. [7] To confirm the diagnosis of GPA, a biopsy was performed in 40 (66.7%) patients: nasal mucosa or paranasal sinuses - in 17 (28.3%) patients, laryngeal formation - in 5 (8.3%) patients, orbital biopsy - in 12 (20.0%), skin - in 1 (2.3%), tympanic cavity - in 1 (2.3%), lungs - in 1 (2.3%), bronchi - in 1 (2.3%), conjunctiva - in 1 (2.3%). The average period from the onset of the disease to the diagnosis and initiation of treatment was 18.3 ± 32.32 months. At the time of the survey, the average duration of GPA in years was 5.7 ± 4.8 (in months 69.6 ± 57.7). 22 out of 60 patients had a local form of HPA (damage to the upper respiratory tract, the organ of hearing and vision). The local form was diagnosed in 7 men and 15 women aged 18 to 70 years (median age 47.5 years). The relapse rate was quite high in both cohorts, but in the prospective cohort it slightly decreased (from 66.2 to 54.2 per 100 patient-years) due to patients with generalized HPA. In the structure of exacerbations of diseases in both cohorts, small relapses prevailed (79.7% in the retrospective and 92.7% in the prospective). The frequency of large relapses in the prospective cohort decreased by about 2 times in patients with both generalized and localized HPA. The data obtained indirectly confirm the effectiveness of more “sparing” immunosuppressive therapy (methotrexate, azathioprine glucocorticosteroids only), which we have begun to use more often in recent years.
