Capecchi Riccardo, Giannese Domenico, Moriconi Diego, Bonadio Angelo G, Pratesi Federico, Croia Cristina, Egidi Maria F, Puxeddu Ilaria, Tavoni Antonio G, Migliorini Paola
Clinical Immunology and Allergy Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
Nephrology, Dialysis and Transplantation Unit, Azienda Ospedaliero Universitaria Pisana, Pisa, Italy.
Front Med (Lausanne). 2021 Mar 31;8:635706. doi: 10.3389/fmed.2021.635706. eCollection 2021.
IgG4-Related Disease (IgG4-RD) is a fibroinflammatory condition characterized by a typical histopathological pattern (dense lymphoplasmacytic infiltrate with prevalent IgG4+ plasma cells and storiform fibrosis), which may involve the kidney both directly (IgG4-related kidney disease, IgG4-RKD) or indirectly, as a consequence of post-renal ureteral obstruction due to retroperitoneal fibrosis (IgG4-RD RF). The most frequent presentation of IgG4-RKD is IgG4-related tubulointerstitial nephritis (TIN), but a glomerular disease can be present, in most of the cases a membranous nephropathy. Albeit steroid-responsive, in some cases renal manifestations may lead to progressive and permanent organ damage. In this review we describe four clinical cases representative of typical and less typical renal manifestations of IgG4-RD, emphasizing a potential, subclinical, early involvement of the kidney in the disease.
IgG4相关疾病（IgG4-RD）是一种纤维炎性疾病，其特征为典型的组织病理学模式（密集的淋巴细胞和浆细胞浸润，以IgG4+浆细胞为主，伴有束状纤维化），可直接累及肾脏（IgG4相关肾病，IgG4-RKD），或因腹膜后纤维化导致肾后输尿管梗阻而间接累及肾脏（IgG4-RD RF）。IgG4-RKD最常见的表现是IgG4相关肾小管间质性肾炎（TIN），但也可能存在肾小球疾病，大多数情况下为膜性肾病。尽管对类固醇有反应，但在某些情况下，肾脏表现可能导致进行性和永久性器官损害。在本综述中，我们描述了四个代表IgG4-RD典型和不太典型肾脏表现的临床病例，强调了肾脏在该疾病中可能存在的亚临床早期受累情况。
