Rituximab for retroperitoneal fibrosis due to IgG4-related disease: A case report and literature review.

Abstract

Retroperitoneal fibrosis (RPF) is a progressive fibroinflammatory disease that can be complicated by urinary obstruction. RPF can be the only manifestation of IgG4-related disease (IgG4-RD). Treatment of IgG4-related RPF is challenging and mostly consists of long-term glucocorticoids leading to significant side effects and treatment intolerance. Recent exploration of the role of rituximab as a B-cell depleting therapy in the treatment of IgG4-RD provides therapeutic potential as a well-tolerated alternative to glucocorticoids. We present a case of IgG4-related RPF for which rituximab was instituted as a steroid-sparing treatment strategy. Following 4 doses, kidney function partially recovered, and the disease went into remission. We discuss the potential merit of rituximab for the treatment of patients with IgG4-related RPF.

• Authors: Mohammad Almeqdadi, Mohammed Al-Dulaimi, Aleksandr Perepletchikov, Kevin Tomera, Bertrand L Jaber
• Journal: Clinical nephrology. Case studies (Clin Nephrol Case Stud) Vol. 6 Pg. 4-10 ( 2018) ISSN: 2196-5293 [Electronic] Germany
• PMID: 29845002 (Publication Type: Case Reports)