Hu Jia-Qi, Jin Zheng-Yi, Yu Yi-Yi, Min Du-Mu, Cai Qing, Gao Jie
Department of Rheumatology and Immunology, The First Affiliated Hospital of Naval Medical University, Shanghai, 200433, China.
Clin Rheumatol. 2025 Feb;44(2):757-766. doi: 10.1007/s10067-024-07276-w. Epub 2025 Jan 4.
Retroperitoneal fibrosis (RPF) is a rare condition marked by inflammation and fibrosis affecting the peritoneal and retroperitoneal soft tissues. In recent years, the identification of IgG4-related diseases has brought to light a significant association with fibrous disorders, including RPF, which were once considered independent. In this comprehensive cohort study, we performed a comparative analysis of the demographic, clinical, laboratory, histopathological, and therapeutic characteristics between patients with IgG4-related RPF and those with idiopathic retroperitoneal fibrosis (iRPF).
We performed a retrospective analysis of 117 patients diagnosed with RPF at the First Affiliated Hospital of Naval Medical University between July 2007 and July 2023.
Demographic, clinical, laboratory, histopathological, and therapeutic characteristics of 70 iRPF patients and 47 IgG4-related patients were systematically compared. The IgG4-related group exhibited an older age of onset, with a predominant occurrence among adult males. Significantly elevated levels of eosinophilia and IgE were observed in the IgG4-related group. Most patients across both groups displayed elevated CRP and ESR levels. Furthermore, at the time of diagnosis, the IgG4-related group had higher serum creatinine and lower levels of complement. The most prevalent clinical manifestation in both groups was flank pain. The proportion of lymphoplasmic infiltration and storiform fibrosis in IgG4-related RPF group was significantly higher. The IgG4-related RPF group had significantly higher IgG4-positive plasma cell count, IgG4/total IgG ratio, and eosinophils count than that in iRPF group.
We conducted a comparative analysis of demographic, clinical, laboratory, histopathological, and therapeutic differences between the iRPF patients and the IgG4-related patients. Clarifying the distinctive characteristics of these two groups will contribute to a better understanding of the condition and facilitate the development of specific treatment strategies tailored to each group. Key Points • Identification of distinct clinical features and outcomes between IgG4-related and iRPF patients in a large retrospective study.
腹膜后纤维化（RPF）是一种罕见疾病，其特征为炎症和纤维化累及腹膜和腹膜后软组织。近年来，IgG4相关疾病的识别揭示了其与包括RPF在内的纤维性疾病之间的显著关联，而这些疾病曾被认为是独立的。在这项综合性队列研究中，我们对IgG4相关RPF患者和特发性腹膜后纤维化（iRPF）患者的人口统计学、临床、实验室、组织病理学和治疗特征进行了比较分析。
我们对2007年7月至2023年7月期间在海军军医大学第一附属医院诊断为RPF的117例患者进行了回顾性分析。
系统比较了70例iRPF患者和47例IgG4相关患者的人口统计学、临床、实验室、组织病理学和治疗特征。IgG4相关组发病年龄较大，以成年男性为主。IgG4相关组嗜酸性粒细胞和IgE水平显著升高。两组大多数患者的CRP和ESR水平均升高。此外，在诊断时，IgG4相关组血清肌酐较高，补体水平较低。两组最常见的临床表现均为胁腹痛。IgG4相关RPF组淋巴细胞浸润和席纹状纤维化的比例显著更高。IgG4相关RPF组的IgG4阳性浆细胞计数、IgG4/总IgG比值和嗜酸性粒细胞计数均显著高于iRPF组。
我们对iRPF患者和IgG4相关患者的人口统计学、临床、实验室、组织病理学和治疗差异进行了比较分析。明确这两组的独特特征将有助于更好地理解该病，并促进针对每组制定特定的治疗策略。要点•在一项大型回顾性研究中识别IgG4相关和iRPF患者之间不同的临床特征和结局。
