Rituximab for idiopathic and IgG4-related retroperitoneal fibrosis.

ABSTRACT: Retroperitoneal fibrosis (RPF) refers to a fibro-inflammatory lesion in the retroperitoneum, often anterolateral to the aorta. Most cases are due to IgG4-related disease (IgG4-RD) or are idiopathic. RPF can lead to severe morbidity. Treatment strategies remain poorly-defined. We evaluated the efficacy and safety of rituximab (RTX) for idiopathic or IgG4-related RPF.We retrospectively reviewed the records of patients who had RPF treated with RTX. Treatment response was determined by assessing changes in both clinical features, including symptoms and laboratory measurements, as well as in the radiographic dimensions of the lesion.Twenty-six patients with IgG4-related (n = 19) or idiopathic RPF (n = 7) were identified. Patients without histopathological evidence of IgG4-RD on either retroperi

SUBMITTER: Wallwork R 

PROVIDER: S-EPMC6211888 | biostudies-literature | 2018 Oct

REPOSITORIES: biostudies-literature