Rossi Giovanni Maria, Rocco Rossana, Accorsi Buttini Eugenia, Marvisi Chiara, Vaglio Augusto
Nephrology Unit, Parma University Hospital, Via Gramsci 14, 43126, Parma, Italy.
Intern Emerg Med. 2017 Apr;12(3):287-299. doi: 10.1007/s11739-016-1599-z. Epub 2017 Jan 9.
Retroperitoneal fibrosis (RPF) is a rare disease characterised by fibrous tissue proliferation in the retroperitoneum, with encasement of the ureters and large vessels of the abdomen as the most destructive of potentially severe complications. It can either be idiopathic, or secondary to infections, malignancies, or the use of certain drugs. The idiopathic form accounts for approximately 75% of the cases, and is usually responsive to immunosuppressive therapy. In recent years, the emergence of a new clinical entity, IgG4-related disease (IgG4-RD), shed light on many fibro-inflammatory disorders once thought to be separate clinical entities, although frequently associated in the so-called multifocal fibrosclerosis. Among these, together with sclerosing pancreatitis and cholangitis, pseudotumour of the orbit, idiopathic mediastinal fibrosis and other conditions, is idiopathic retroperitoneal fibrosis (IRF). Both IRF and IgG4-RD can be associated with a wide variety of disorders, usually governed by immune-mediated (and particularly auto-immune) mechanisms. In our review, we discuss the clinical and therapeutic challenges IRF presents to the internist, as well as the meaning of its recent inclusion in the IgG4-RD spectrum from a clinical practice standpoint.
腹膜后纤维化（RPF）是一种罕见疾病，其特征为腹膜后纤维组织增生，其中输尿管和腹部大血管受累是最具破坏性的潜在严重并发症。它可以是特发性的，也可以继发于感染、恶性肿瘤或某些药物的使用。特发性形式约占病例的75%，通常对免疫抑制治疗有反应。近年来，一种新的临床实体——IgG4相关性疾病（IgG4-RD）的出现，为许多曾被认为是独立临床实体的纤维炎性疾病带来了新的认识，尽管这些疾病在所谓的多灶性纤维硬化症中经常相关联。其中，与硬化性胰腺炎和胆管炎、眼眶假瘤、特发性纵隔纤维化及其他病症一起的，是特发性腹膜后纤维化（IRF）。IRF和IgG4-RD都可能与多种疾病相关，通常由免疫介导（尤其是自身免疫）机制所主导。在我们的综述中，我们讨论了IRF给内科医生带来的临床和治疗挑战，以及从临床实践角度看其最近被纳入IgG4-RD范畴的意义。
