Department of Urology, University Hospitals Leuven , Leuven, Belgium.
Department of Internal Medicine, Nephrology and Renal Transplantation Research Group , Leuven, Belgium.
Acta Clin Belg. 2020 Aug;75(4):239-244. doi: 10.1080/17843286.2019.1609152. Epub 2019 Apr 29.
Idiopathic retroperitoneal fibrosis (iRPF) is a rare fibro-inflammatory disease, characterized by inflammation of the abdominal aorta and its surrounding structures. The exact pathophysiology remains unclear. Diagnosis is often troublesome due to the non-specific and highly variable clinical presentation. Standardized treatment protocols are lacking.
This article presents a review on iRPF, addressing clinical and diagnostic modalities as well as its pathophysiology and the possible inclusion within the IgG4-related disease (IgG4-RD) spectrum. Finally, a diagnostic-therapeutic algorithm for a standardized approach to iRPF is proposed.
The PubMed Internet database was searched. Articles were selected based on the relevance of abstract, article type and impact of the journal.
iRPF and IgG4-RD share a common autoimmune aetiology. Diagnostics are multimodal and based on imaging. Ruling out malignancy should be of primary concern. Complications are mostly of renal or vascular origin due to compression of retroperitoneal structures. Corticosteroids remain the first-line treatment regimen and are mostly successful, but evidence supporting alternative immunosuppressive and anti-inflammatory treatments is growing. Long-term therapy, as well as follow-up, is paramount in this chronic and often relapsing disease.
特发性腹膜后纤维化（iRPF）是一种罕见的纤维炎症性疾病，其特征为腹主动脉及其周围结构的炎症。确切的病理生理学仍然不清楚。由于非特异性和高度可变的临床表现，诊断常常很麻烦。缺乏标准化的治疗方案。
本文回顾了 iRPF，介绍了其临床和诊断方法，以及其病理生理学和可能包含在 IgG4 相关疾病（IgG4-RD）谱中的情况。最后，提出了一种 iRPF 标准化诊断-治疗方案的算法。
通过互联网 PubMed 数据库进行检索。根据摘要、文章类型和期刊的影响选择相关文章。
iRPF 和 IgG4-RD 具有共同的自身免疫病因。诊断是多模式的，基于影像学。排除恶性肿瘤应是首要关注的问题。并发症主要源于腹膜后结构受压引起的肾脏或血管病变。皮质类固醇仍然是一线治疗方案，且大多有效，但支持替代免疫抑制和抗炎治疗的证据正在增加。在这种慢性且常复发的疾病中，长期治疗和随访至关重要。
