AB0933 Retroperitoneal Fibrosis, Igg4-Related Diseases and Cardiovascular Fibrosclerosis

Background The diagnosis of cardiovascular fibrosclerosis (CF), become more feasible parallel to technologic improvements in imaging modalities. CF may manifest as idiopathic retroperitoneal fibrosis (RPF), inflammatory aortic aneurysm (IAA), inflammatory periarteritis, and inflammatory pericarditis.1 A certain fraction, but not all, of CF may have a link with immunoglobulin-G4 (IgG4)-related sclerosing disease (IgG4-SD). Most of the data depends on case reports/series. Objectives This study is aimed to investigate the experience our rheumatology clinic and increase the awareness of physicians about this concept. Methods Our database was screened regarding manifestations of CF. Demographic, clinical characteristics and imaging studies in addition to IgG4 levels were recorded. Patients fulfilling criterias of any connective tissue diseases and/or vasculitis were excluded. Also the patients are divided into two groups a (IgG4 elevated or normal range). Vascular and extravascular involvement characteristics of patients are compared. Results Totally 16 patients (Male/Female: 10/6) were included into the study. Mean age was 53.6±9.2 years. Most of the patients had RPF. Distribution of involvements was shown in Table 1. Serum IgG4 levels were measured in all of the patients. Nine (56.3%) of patients had elevated IgG4 levels. Patients with elevated IgG4 levels had more systemic involvements (periorbital and lymphadenopathy involvements were statistically significant-Table). In all of the periarteritis cases, coronary artery had been involved. One of the patients with coronary arteritis had normal IgG4 level. Even though pulse corticosteroid therapy, this patient had coronary artery bypass graft because of resistant angina pectoris and elevated cardiac enzymes (Fig. 1). Table 1. Distribution of organ involvements and comparison of patient groups In all group Elevated IgG4 levels Normal IgG4 levels p n (%) (n=9) (n=7) Retroperitoneal Fibrosis 12 (75.0) 5 (55.6) 7 (100) 0.042 Aortitis 6 (37.5) 5 (55.6) 1 (14.3) 0.091 IAA 2 (12.5) 2 (22.2) 0 0.182 Inflammatory periarteritis 4 (25.0) 3 (33.3) 1 (14.3) 0.383 Pericarditis/pericardial fibrosis 4 (25.0) 3 (33.3) 1 (14.3) 0.383 Pleural Fibrosis 2 (12.5) 2 (22.2) 0 0.182 Lymphadenopathy 4 (25.0) 4 (44.4) 0 0.042 Pancreas involvement 3 (18.8) 2 (22.2) 1 (14.3) 0.687 Periorbital involvement 4 (25.0) 4 (44.4) 0 0.042 Figure 1 Conclusions Cardiovascular fibrosclerosis is an emerging concept in rheumatology. Patients with RPF sh