Abstract
Retroperitoneal fibrosis (RPF) is an uncommon condition characterized by inflammation and fibrosis in the retroperitoneal space. More than two-thirds of RPF are idiopathic, with the remaining stemed from a variety of secondary causes. It was suggested that IgG4-related RPF is a secondary form of RPF. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-related RPF and IRPF in a large Chinese cohort. We retrospectively reviewed the medical records of 132 RPF patients diagnosed at Peking University People’s Hospital between March 2010 and March 2018. Among the 132 patients, the mean age at disease onset was 54.8 years. IgG4-related RPF group showed greater male predominance compared to IRPF group. IgG4-related RPF patients showed a longer interval between symptom onset and diagnosis, and allergic diseases were more common in this group. Sixty-four patients (48.4%) had lower back pain, which was more common in IRPF group than that in IgG4-related RPF patients. In terms of organ involvement, although 42 of 47 patients (89.3%) with IgG4-related RPF had other organ involvement, there were no patients in the IRPF group with other organ involvement. In addition, the serum IgG4 level, elevated eosinophils counts and IgE level were significantly higher in IgG4-related RPF patients. We described the demographic, clinical and laboratory differences between IgG4-related RPF and IRPF patients, indicating their potential differences in pathogenesis, which was of great importance to diagnose and manage the two phenotypes.
Highlights
Retroperitoneal fibrosis (RPF) is a rare disorder characterized by the presence of chronic inflammation and fibrosis in the retroperitoneal space [1]
Patients were divided into two groups, immunoglobulin G4 (IgG4)-related RPF and idiopathic retroperitoneal fibrosis (IRPF), based on the comprehensive diagnostic criteria published by Umehara et al [15] in 2011
Among the 132 cases in our cohort, 47 patients (35.6%) were classified as having IgG4-related RPF (19 definite, 5probable, 23possible), and 85 patients were classified as having IRPF
Summary
Retroperitoneal fibrosis (RPF) is a rare disorder characterized by the presence of chronic inflammation and fibrosis in the retroperitoneal space [1]. IgG4-RD consists of fibro-inflammatory diseases that affect a variety of structures (e.g, pancreas, biliary tract, lymph nodes, retroperitoneum). It is featured with lympho-plasmacytic inflammation, infiltration by IgG4+ plasma cells and irregular and distinct fibrosis [5, 6]. Choi [14] found that the clinical and laboratory characteristics of IgG4-related RPF are similar to those of IRPF, except for male predominance, older age, and higher incidence of postrenal AKI in IgG4-related RPF. We conducted this study to compare the demographic, clinical and laboratory characteristics of IgG4-related RPF and IRPF patients in a large Chinese cohort. Defining the characteristics of these two groups might help facilitate advanced recognition of this condition, identify its risk factors and develop specific treatment strategies for each cluster
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