Background/Purpose: IgG4-related disease is a recently recognised inflammatory disease of unkown etiology, often seen in men over the age of 50 and may affect many organs and systems with elevated serum IgG4 levels and typical histopathological features.The aim of this study is to determine the demographic and clinical characteristics of patients with IgG4-related disease

Methods: Patients diagnosed as having Ig-G4-related disease by their typical histopathological findings and imaging features and/or increased serum IgG4 concentrations ( > 135 mg / dl) from two university hospital in Izmir were included in the study.

Results: There were 53 patients with a mean age of 51.49 yrs (69.8% male). The most common involvement was retroperitoneal fibrosis (54.7%), followed by the cardiovascular system (CVS) involvement (45.3%) (Table 1). While 22 patients had at least two organ involvement, the most common association was retroperitoneal fibrosis and CVS involvement (15 patients). Serum IgG4 levels were studied in 36 patients (67.9%) and found to be higher levels in 20 patients. (55.5%) (Table 2). In 44 patients (83%), acute phase reactants (APRs) were increased at the time of the diagnosis. There was no correlation between the extent of the disease and serum IgG4 levels and initial erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) values. 28 patients (52.8%) were diagnosed by imaging, 9 (17%) by imaging and IgG4 elevation, 5 (9.4%) by imaging and histopathology, 10 (18.9%) by imaging, histopathology and IgG4 elevation and 1 patient (1.9%) by only histopathology.  The most commonly (58.5%) used imaging method for diagnosis was computed tomography (CT). All the patients used initial glucocorticoid treatment.4 patients (7.5%) recieved only glucocorticoid,others were underwent the following treatments combined with glucocorticoid: azathioprine (AZA) (60.4%); methotrexate (mtx) (11.3%), rituximab (RTX) + AZA (9.4%), mtx + AZA (5.7%), RTX (3.8%) and infliximab (1.9%).  In the follow-up, a significant decrease in acute phase reactants was found in 62% of the patients at their last visits. While 27.3% of the patients had complete remission, 36.4% had partial remission, 20.5% had stable course, 13.6% had progression in the disease and 2.3% had recurrence.  In 18 patients (64.3%) out of 28 patients who were in partial or complete remission, remission was achieved by using glucocorticoid and AZA combination treatment.

Conclusion: In conclusion, we have described a considerably large serie of patients with IgG4-related disease from Turkey. The results of the study suggested that AZA and glucocorticoid combination treatment was commonly used in Turkish patients with IgG4-related disease and it might be a good treatment option to achieve remission.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/azathioprine-and-glucocorticoid-combination-might-be-a-good-treatment-option-to-achieve-remission-in-patients-with-igg4-related-disease/