Background The epidemiological findings of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) differ geographically. Until now, there has been no study about the prevalence of AAV in Turkey.
Objectives In our study, we aimed to evaluate the prevalence of AAV in northeastern part of Turkey.
Methods Patients were diagnosed in our medical center in Edirne. Our hospital has been the only tertiary referral center for rheumatic diseases for a mixed rural and urban population of 616,000 people (˃16 years). We retrospectively recorded down the general features of patients with AAV diagnosed at our center within the last 10 years. The incidence rates per 100000 aged ≥16 were calculated. Also we evaluated clinical features of patients with AAV.
Results There were 20 patients with Wegener granulomatosis (WG); 7 with microscopic polyangiitis (MPA); and 4 with Churg-Strauss syndrome (CSS). During the study period, the annual incidence rate was 5.03/million for all AAV. The annual incidence of AAV in women was 3.32/million, and in men was 6.66/million. The annual incidence for WG for subjects 15 years of age or older was calculated as 3.24 per million, and the incidence for MPA was calculated as 1.13 per million, and the incidence of CSS was 0.65 per million. In addition, we had previously investigated the prevalences of various rheumatic diseases including vasculitis in the Havsa district and its urban areas, in northwestern part of Turkey (1). In our population-based study in this same area we did not diagnose any AAV patient.
The median age at diagnosis was 57 (29–76) years for all patients and 52.5 (30-69) years for WG patients.
In patients with WG, 19 (95%) tested positive for PR3, cytoplasmic ANCA at the time of diagnosis. In patients with MPA or CSS, 9 (81.8%) tested positive for MPO, perinuclear ANCA and 1 (9.1%) tested positive for PR3, cytoplasmic ANCA. RF positivitvity was detected in 6 (30%) WG patients and antinuclear antibody was positive in 6 (54.5%) patients with MPA or CSS. Renal disease was detected in 11 (55%) patients with WG and 3 (27.3%) patients with MPA or CSS. End-stage renal disease developed in 6 (30%) WG patients and in 1 (9.1%) patient with MPA or CSS. Pulmonary involvement was defined in 16 (80%) patients with WG (3 of them alveolar hemorrhage) and in 7 (63.6%) patients with MPA or CSS. In patients with WG, there were 7 (35%) with paranasal sinus involvement and 6 (30%) with eye involvement. Severe CNS involvement (2 WG and 1 MPA cases) and peripheral neuropathy (1 WG and 2 CSS patients) were seen rarely. At the time of diagnosis, physical examination revealed arthritis in 8 (40%) WG patients and in 3 (27.3%) MPA or CSS patients. Oral ulcers were seen in 6 (50%) WG and saddle nose deformity developed in only 1 WG patients. The median follow-up time for all AAV patients was 42 months (4-120). 5 patients with AAV were died (3 of them WG and 2 of them MPA). All dead patients were male. 5 and 10-years survivals of patients with AAV were 81.5% and 70% respectively.
Conclusions In northwestern Turkey, the annual incidences of all AAV and WG were lower than in western countries.
References Cakır N, Pamuk ÖN, Derviş E, Imeryüz N, Uslu H, Benian Ö, Elelçi E, Erdem G, Sarvan FO, Senocak M. The prevalences of some rheumatic diseases in western Turkey: Havsa study. Rheumatol Int 2012;32(4):895-908.
Disclosure of Interest None Declared