Use of rituximab in idiopathic retroperitoneal fibrosis

Abstract

Background: Retroperitoneal fibrosis (RPF) is characterized by the proliferation of fibrous tissue in the retroperitoneum. The majority of RPF cases are due to idiopathic or IgG4-related disease. Recent studies on IgG4related disease have shown rituximab to be an effective treatment. The current first-line treatment for idiopathic RPF (iRPF) is glucocorticoid therapy. Relapse rates vary widely in the literature, and DMARDs remain poorly studied. We sought to evaluate the efficacy of rituximab in idiopathic RPF by quantifying changes in iRPF diameter on imaging pre-and post-rituximab therapy and response by lab parameters in 10 iRPF patients. Methods: We selected 10 patients diagnosed with iRPF and previously treated with rituximab (1000 mg) in two doses approximately 2 weeks apart. Pre-and post-therapy contrast enhanced cross-sectional abdomen and pelvis imaging were compared. In all patients, the thickest portion of the peri-aortic disease was measured in the axial and coronal planes. The presence of acute or long standing back pressure related renal findings were documented. Details of clinical visits including patient demographics and laboratory evaluations were collected pre-and posttherapy. Statistical analysis was performed using a Wilcoxon signed rank test. Results: The RPF diameter around the aorta before and after therapy decreased from a mean of 15.9 ± 4.9 mm to 10.6 ± 6.1 mm, respectively (p < 0.01). The craniocaudal iRPF mean length decreased from 108.6 mm ± 40.4 mm to 90.6 mm ± 45.9 mm (p = 0.02). Conclusion: A comparison of pre and post-rituximab imaging studies revealed a statistically significant decrease in iRPF diameter following treatment with rituximab.

Rituximab in the management of retroperitoneal fibrosis: A single tertiary rheumatology care center experience

“…Urban et al 13 reported that craniocaudal length and SUVmax values significantly decreased following the rituximab therapy. In a study by Boyeva et al 14 the craniocaudal diameter and periaortic thickness of the fibrotic mass measured in magnetic resonance imaging and CT studies were shown to be significantly reduced after rituximab therapy. Our current results and the literature data suggest that rituximab may be an effective treatment in RPF patients with metabolically active disease despite the use of prednisolone, and the previous data indicate that these cases respond more to immunosuppressive treatments.…”

“…We briefly summarize the two previously published retrospective reports on rituximab use in RPF in Table 4. 13,14 Hydronephrosis is the most serious renal complication of RPF and preserving renal function is one of the main goals of treatment in these patients. Although the indwelling ureteral stents relieve hydronephrosis, these stents may lead to various complications such as infection and bleeding.…”

Therapy of immunoglonuline IgG4 related disease (IgG4-RD)

“…Počet léčebných odpovědí byl v obou ramenech této studie stejný, ale v případě použití cyklofosfamidu s glukokortikoidy měla léčebná odpověď delší trvání než pří kombinaci glukokortikoidů s mykofenolát mofetilem (21). a podobné výsledky byly dosaženy u retroperitonální fibrózy (52)(53)(54)(55)(56)(57)(58)(59)(60)(61)(62)(63)(64)(65)(66)(67).…”

When idiopathic retroperitoneal fibrosis mimics Castleman disease: a challenging differential diagnosis

“…Currently, corticosteroids represent the first-line treatment for I-RPF 1. In refractory cases or when contraindications to glucocorticoid therapy are present, the use of rituximab represents a valid strategy, as it has been shown to be associated with a statistically significant shrinkage of RPF on post-treatment imaging, with a favourable side effect profile 5. Future studies are needed to further investigate the different therapeutic options in the systemic treatment of I-RPF.…”