Authors: ÖMER KARADAĞ, ABDULSAMET ERDEN, EMİNE ARZU AYHAN, ERTUĞRUL ÇAĞRI BÖLEK, UMUT KALYONCU, BERKAN ARMAĞAN, ALPER SARI, LEVENT KILIÇ, ALİ AKDOĞAN, TUNCAY HAZIROLAN, BÜLENT AKDOĞAN, ŞAZİYE ŞULE APRAŞ BİLGEN, DİLEK BAYDAR, SEDAT KİRAZ, ALİ İHSAN ERTENLİ
Abstract: Background/aim: Since the majority of the IgG4-related disease (IgG4-RD) patients in the literature are from the Far East and the United States, there is a lack of large series from other parts of the world. We aimed to identify the clinical characteristics and outcome of Turkish IgG4-RD patients from a tertiary center.Materials and methods: Fifty-two patients classified as having definite IgG4-RD according to comprehensive diagnostic criteria were included in the study. Patients not fulfilling the definite criteria due to lack of pathologic specimen and/or serum IgG4 levels were excluded (n = 47). Clinical, laboratory, and histopathological features and treatment approaches were analyzed.Results: Median age at diagnosis was 51.1 years and sex predominance was not observed (male/female: 26/26). Median follow-up duration was 18 (IQR 25-75: 8-35) months. Retroperitoneal fibrosis was the most frequent presentation. Twenty-four (46.1%) patients had localized involvement. Corticosteroids were the mainstay of treatment (92.5%). Rituximab had been used for cases resistant to previous treatment or with relapses in 19 (47.5%) patients. A complete response was achieved in 52.5% and partial response (<50% regression) in 40%. Conclusion: This large and first cohort of IgG4-RD patients from Turkey showed similar clinical features to European cohorts, except for the male predominance in previous cohorts. Corticosteroids and rituximab are effective in IgG4-RD but there is still uncertainty about the usage of corticosteroid-sparing agents.
Keywords: IgG4-related disease, retroperitoneal fibrosis, coronary periarteritis, corticosteroids, immunosuppressive agents, rituximab
Full Text: PDF