SUNY Upstate, Syracuse, NY, USA.
UHS Wilson Medical Center, Johnson City, NY, USA.
J Investig Med High Impact Case Rep. 2021 Jan-Dec;9:23247096211022487. doi: 10.1177/23247096211022487.
Retroperitoneal fibrosis is caused by the replacement of normal retroperitoneal tissue with fibrosis. The majority of the cases are idiopathic, but some secondary causes include malignancy, infection, drugs, and radiotherapy. Immunoglobulin G-4 (IgG-4) related disease is a relatively newer disease and one of the rarer causes of retroperitoneal fibrosis. It usually involves the pancreas, lungs, kidneys, aorta lacrimal and salivary glands, or extrapancreatic bile duct. Elevated serum IgG-4 is the biomarker of the disease and its levels correlate with disease activity. High-dose glucocorticoid is the treatment of choice.
腹膜后纤维化是由正常腹膜后组织被纤维组织取代引起的。大多数病例为特发性，但一些继发性病因包括恶性肿瘤、感染、药物和放射治疗。免疫球蛋白 G-4（IgG-4）相关疾病是一种相对较新的疾病，也是腹膜后纤维化较罕见的病因之一。它通常累及胰腺、肺、肾脏、主动脉泪腺和唾液腺或胰外胆管。血清 IgG-4 升高是该病的生物标志物，其水平与疾病活动度相关。大剂量糖皮质激素是治疗的首选。
