Last update 01 Nov 2024

Retroperitoneal Fibrosis

Last update 01 Nov 2024

Basic Info

Synonyms

Disease, Ormond, Disease, Ormond's, FIBROSIS RETROPERITONEAL

+ [45]

Introduction

A slowly progressive condition of unknown etiology, characterized by deposition of fibrous tissue in the retroperitoneal space compressing the ureters, great vessels, bile duct, and other structures. When associated with abdominal aortic aneurysm, it may be called chronic periaortitis or inflammatory perianeurysmal fibrosis.

Drugs associated with Retroperitoneal Fibrosis

Lenalidomide

Target

CK1α x CRBN x IFNγ x IKZF1 x IKZF3 x IL-2 x IL-6 x TNF-α

Mechanism

CK1α inhibitors [+7]

Active Org.

Bristol-Myers Squibb Pharma EEIG [+31]

Originator Org.

Celgene Corp.

Active Indication

Marginal Zone B-Cell Lymphoma [+65]

Inactive Indication

Aase Smith Syndrome 2 [+98]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

First Approval Date27 Dec 2005

Rituximab

Target

CD20

Mechanism

CD20 inhibitors [+3]

Active Org.

National Cancer Institute [+27]

Originator Org.

Genentech, Inc.

Active Indication

Cardiac transplant rejection [+109]

Inactive Indication

Acute Graft Versus Host Disease [+124]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

First Approval Date26 Nov 1997

NovRF

Target-

Mechanism-

Active Org.

NovMetaPharma Co., Ltd.

Originator Org.

NovMetaPharma Co., Ltd.

Active Indication

Retroperitoneal Fibrosis

Inactive Indication-

Drug Highest PhasePhase 1/2

First Approval Ctry. / Loc.-

First Approval Date20 Jan 1800

Clinical Trials associated with Retroperitoneal Fibrosis

NCT05428826 / RecruitingPhase 4IIT

Early Discontinuation of Steroid Treatment in Negative FDG-PET/CT Patients With Idiopathic Retroperitoneal Fibrosis. A Prospective Multicentric Study

Adult patients with a diagnosis of idiopathic retroperitoneal fibrosis Prospective multicentric cohort study Intervention : administration of prednisone during 9 to 21 months at 1mg/kg/day at inclusion.

Start Date25 Nov 2022

Sponsor / Collaborator

Assistance Publique des Hôpitaux de Paris SA

ChiCTR2200064232 / SuspendedEarly Phase 1IIT

The effectiveness and safety study of Pirfenidone in therapy of idiopathic retroperitoneal fibrosis

Start Date30 Sep 2022

Sponsor / Collaborator-

NCT05133895 / CompletedNot ApplicableIIT

Pilot Study of Tocilizumab Monotherapy for Active Chronic Periaortitis

This is a prospective study to investigate the treatment response of Tocilizumab on patients with active chronic periaortitis (CP).
Methods: patients with a definite or possible diagnosis of CP at acute active stage were enrolled for this study and accepted Tocilizumab monotherapy for 3 months.
Endpoints: The primary endpoint is to investigate the treatment response of Tocilizumab; the secondary endpoints include the improvement of inflammatory markers, the frequency of adverse events.

Start Date15 Jul 2020

Sponsor / Collaborator

Peking Union Medical College Hospital

100 Clinical Results associated with Retroperitoneal Fibrosis

100 Translational Medicine associated with Retroperitoneal Fibrosis

0 Patents (Medical) associated with Retroperitoneal Fibrosis

3,721

Literatures (Medical) associated with Retroperitoneal Fibrosis

18 Oct 2024·Asian journal of surgery

A case of diffuse large B-cell lymphoma surrounding the external iliac vessels misdiagnosed by ultrasound as retroperitoneal fibrosis.

Letter

Author: Zhu, Changfu ; Zhou, Jianan ; An, Qi

18 Oct 2024·Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences

[Etiological analysis of hydronephrosis in adults: A single-center cross-sectional study].

Article

Author: He, Yuhui ; Xu, Yangyang ; Huang, Yanbo ; Wang, Haiju ; Guan, Hua ; Li, Zhihua ; Chen, Silu ; Li, Xuesong ; Wu, Yucai

OBJECTIVETo investigate the etiological distribution of hydronephrosis caused by upper urinary tract obstruction in adult patients and to improve the diagnostic accuracy for this condition.METHODSThe clinical information of adult patients with newly diagnosed hydronephrosis in Upper Urinary Tract Repair Outpatient Clinic of Peking University First Hospital from May 2020 to May 2021 were prospectively and continuously collected. Patients with ureteral calculi or upper urinary tract tumor were excluded. A total of 767 patients were involved. The underlying causes of upper urinary tract obstruction were identified by senior urological surgeons according to symptoms, medical history, physical examination, and a range of diagnostic imaging techniques including ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), retrograde pyelography, antegrade pyelography, radionuclide renogram and ureteroscopy.RESULTSAmong the 767 patients, 359(46.8%) were male and 408(53.2%) were female. The median age of these patients was 37 years (range, 14-84 years). Hydronephrosis was observed at left-sided in 357 cases(46.6%), right-sided in 251 cases(32.7%), and bilateral in 159 cases(20.7%). The causes of hydronephrosis were classified as follows: (1) Non-iatrogenic factors were found in 464 cases (60.5%). These included urinary malformations in 355 cases(76.5%), infection in 29 cases(6.3%), pelvic lipomatosis and/or cystitis glandularis in 23 cases(5.0%), ureteral endometriosis in 18 cases(3.9%), retroperitoneal fibrosis in 15 cases(3.2%), trauma in 7 cases(1.5%) and other non-iatrogenic factors in 12 cases(2.6%). Some of these patients had multiple non-iatrogenic causes. Among the 355 cases with urinary system malformations, 252 cases (71.0%) had ureteropelvic junction obstruction. (2) Iatrogenic ureteral injuries accounted for 210 cases (27.4%), including 112 cases(53.3%) of urological surgical injuries, 51 cases(24.3%) of radiotherapy for malignant tumor related injuries, 34 cases(16.2%) of gynecological and obstetrical surgical injuries, and 13 cases(6.2%) of general surgical injuries. (3) The cause of hydronephrosis remained unknown in 93 cases(12.1%).CONCLUSIONHydronephrosis in adults due to upper urinary tract obstruction has a diverse range of causes, with urinary malformations and iatrogenic ureteral injuries being significant contributors. Urological surgeon involved in upper urinary tract reconstruction should be familiar with these potential causes to facilitate accurate diagnosis and effective treatment.

02 Oct 2024·Scandinavian Journal of Gastroenterology

New insights into predictors of autoimmune pancreatitis relapse after steroid therapy

Article

Author: Setoguchi, Keigo ; Matsuda, Takahisa ; Chiba, Kazuro ; Igarashi, Yoshinori ; Kamisawa, Terumi ; Tabata, Hiroki ; Ujita, Wataru ; Nakahodo, Jun

OBJECTIVESWhile autoimmune pancreatitis (AIP) responds well to steroid therapy, the high relapse rate in type 1 AIP remains a critical problem. The present study examined predictors of relapse of type 1 AIP following steroid therapy.MATERIALS AND METHODSNine factors potentially predictive of relapse were analyzed in 81 AIP patients receiving steroid therapy with follow-up ≥ 12 months. The rate of serum IgG4 decrease following steroid therapy was calculated by dividing the difference between serum IgG4 values before and at two months after the start of steroid by the IgG4 value before steroid.RESULTSA relapse occurred in 11 patients (13.5%) during a median of 38 months. Multivariate analysis revealed that the presence of IgG4-related retroperitoneal fibrosis (HR: 5.59; 95% CI: 1.42-22.0; p = 0.014) and the low rate of serum IgG4 decrease after steroid therapy (HR: 0.048; 95% CI: 0.005-0.46; p = 0.008) were significant, independent predictors of AIP relapse. The cut-off value based on receiver operating characteristic curve data for the rate of serum IgG4 decrease before and at two months after steroid therapy distinguishing patients with and without a relapse was 0.65. Using this cut-off value, the area under the curve, sensitivity, and specificity were found to be 0.63, 0.73, and 0.60, respectively.CONCLUSIONThe low rate of serum IgG4 decrease after the start of steroid therapy and the presence of IgG4-related retroperitoneal fibrosis were predictive of type 1 AIP relapse. Cautious, gradual tapering of steroid dosage and longer maintenance therapy are recommended for patients with these factors.

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Retroperitoneal Fibrosis

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