Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing, China.
Department of Rheumatology and Immunology, Tengzhou Central People's Hospital, Tengzhou, China.
PLoS One. 2021 Feb 18;16(2):e0245601. doi: 10.1371/journal.pone.0245601. eCollection 2021.
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.
腹膜后纤维化 (RPF) 是一种罕见的疾病，其特征为腹膜后腔的炎症和纤维化。超过三分之二的 RPF 为特发性，其余则由多种继发性原因引起。有人提出 IgG4 相关的 RPF 是 RPF 的一种继发性形式。我们进行了这项研究，旨在比较 IgG4 相关的 RPF 和特发性腹膜后纤维化（IRPF）在中国大样本中的详细人口统计学、临床和实验室特征。我们回顾性地分析了 2010 年 3 月至 2018 年 3 月期间在北京大学人民医院诊断的 132 例 RPF 患者的病历。在 132 例患者中，发病时的平均年龄为 54.8 岁。与 IRPF 组相比，IgG4 相关 RPF 组中男性患者更为多见。IgG4 相关 RPF 患者从症状出现到确诊的时间间隔更长，且该组更常见过敏性疾病。64 例（48.4%）患者有腰痛，在 IRPF 组中比 IgG4 相关 RPF 患者更为常见。在器官受累方面，尽管 47 例 IgG4 相关 RPF 患者中有 42 例（89.3%）存在其他器官受累，但在 IRPF 组中没有患者存在其他器官受累。此外，IgG4 相关 RPF 患者的血清 IgG4 水平、嗜酸性粒细胞计数和 IgE 水平显著升高。我们描述了 IgG4 相关 RPF 和 IRPF 患者之间的人口统计学、临床和实验室差异，表明它们在发病机制上可能存在差异，这对于诊断和管理这两种表型具有重要意义。
