Sica Antonello, Casale Beniamino, Spada Alessandro, Teresa Di Dato Maria, Sagnelli Caterina, Calogero Armando, Buonavolontà Pietro, Salzano Anna, Martinelli Erika, Saracco Elisabetta, Troiani Teresa, Anna Dodaro Concetta, Tammaro Dario, Luisa De Rimini Maria, Ciardiello Fortunato, Papa Alfonso
Department of Precision Medicine, University of Campania Luigi Vanvitelli, Naples, Italy.
Department of Pneumology and Tisiology, AO Dei Colli - V. Monaldi, Naples, Italy.
Open Med (Wars). 2019 Dec 26;15:22-26. doi: 10.1515/med-2020-0005. eCollection 2018.
Retroperitoneal fibrosis is a connective disease of the auto-inflammatory/auto-immune type of the retroperitoneum with unknown etiology and pathological mechanism. The manifestations of the pathology can be local or systemic. Amongst the local symptoms, the dull and constant pain in the hips, back or abdomen is the most frequent. We report here a case of a 47-year-old woman, whose pathogenic mechanism could be related to an "IgG4-related disease" disorder as suggested by an increased serum level of this subclass of IgG and the positive immunohistochemistry. The diagnosis is not easy, because this pathology generates masses; adenomegalies with retro peritoneal development, that makes it similar to lymphomas or metastases from ovarian tumors.
腹膜后纤维化是一种发生于腹膜后的、病因及病理机制不明的自身炎症/自身免疫性结缔组织病。其病理表现可为局部性或全身性。在局部症状中，髋部、背部或腹部的钝痛及持续性疼痛最为常见。我们在此报告一例47岁女性病例，其发病机制可能与“IgG4相关性疾病”紊乱有关，依据是该亚类IgG血清水平升高及免疫组化呈阳性。该疾病诊断并不容易，因为这种病理改变会形成肿块；伴有腹膜后发展的淋巴结肿大，这使其类似于淋巴瘤或卵巢肿瘤转移。
