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Last update 23 Jan 2025

Retroperitoneal Fibrosis

Last update 23 Jan 2025

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 IKZF1 x IKZF3

Mechanism

CK1α inhibitors [+3]

Active Org.

Incyte Corp. [+30]

Originator Org.

Celgene Corp.

Active Indication

Marginal Zone B-Cell Lymphoma [+63]

Inactive Indication

Aase Smith Syndrome 2 [+110]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

United States

First Approval Date27 Dec 2005

Rituximab

Target

CD20

Mechanism

CD20 inhibitors [+3]

Active Org.

National Cancer Institute [+29]

Originator Org.

Genentech, Inc.

Active Indication

Cardiac transplant rejection [+107]

Inactive Indication

Acute Graft Versus Host Disease [+135]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

United States

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

ChiCTR2400094419

/ SuspendedNot ApplicableIIT

A Prospective Cohort Study on the Efficacy of Tamoxifen Combined with Glucocorticoids Versus Glucocorticoid Monotherapy in Treating Idiopathic Retroperitoneal Fibrosis

Start Date01 Jan 2025

Sponsor / Collaborator-

NCT06741423

/ Active, not recruitingNot ApplicableIIT

Distinguishing Retroperitoneal Fibrosis and Sarcoma from Other Retroperitoneal Diseases on CT Scans Via an Extended-Radiomics Approach: a Multi-Centric, International Retrospective Analysis.

A retrospective study utilizing archived CT scans of patients diagnosed with retroperitoneal fibrosis, sarcoma or other malignancies (i.e. lymphoma, germ cell tumors, metastasis, infections, ganglioneuromas) in order to implement a radiomics algorithm which is able to differentiate between these malignancies.

Start Date01 Nov 2023

Sponsor / Collaborator

Heidelberg University, U.S.

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

100 Clinical Results associated with Retroperitoneal Fibrosis

100 Translational Medicine associated with Retroperitoneal Fibrosis

0 Patents (Medical) associated with Retroperitoneal Fibrosis

3,767

Literatures (Medical) associated with Retroperitoneal Fibrosis

01 Jan 2025·Disease-a-Month

Erdheim–Chester disease: Comprehensive insights from genetic mutations to clinical manifestations and therapeutic advances

Article

Author: Kumar, Anand ; Srivastava, Siddhi ; Singh, Alpana ; Chaudhary, Rishabh ; Mishra, Vikas ; Srivastava, Sukriti ; Agarwal, Vipul ; Rehman, Mujeeba ; Kaushik, Arjun Singh

Erdheim-Chester disease (ECD) is an extremely rare non-Langerhans cell disorder that is believed to include both inflammatory and neoplastic characteristics. It is caused due to genetic mutations in proto-oncogenes like BRAF and MEK, while immunological pathways have an essential role in the onset and progression of the disease. Despite its rarity, ECD poses significant diagnostic and therapeutic challenges due to its heterogeneous clinical presentation and limited understanding of its underlying pathophysiology. Multiple organs can be affected, with the most frequent being long bones, central nervous system and retro-orbital abnormalities, pericardial and myocardial infiltration, interstitial lung disease, retroperitoneal fibrosis, and large blood vessel aberrations. Here, in this review, we comprehensively underline the current knowledge of ECD, including its epidemiology, clinical manifestations, genetics, pathophysiology, diagnostic modalities, and treatment options. By synthesizing existing literature and highlighting areas of ongoing research, this review aims to provide clinicians and researchers with a comprehensive understanding of ECD and guide future directions for improved patient care and outcomes.

06 Dec 2024·Hematology

IgG4-related disease for the hematologist

Review

Author: Chen, Luke Y. C.

AbstractImmunoglobulin G4–related disease (IgG4-RD) is an immune-mediated disease with many important manifestations in hematopoietic and lymphoid tissue. IgG4 is the least naturally abundant IgG subclass, and the hallmark feature of IgG4-RD is markedly increased IgG4-positive plasma cells (with an IgG4 to IgG ratio >40%) in affected tissue, along with elevated polyclonal serum IgG and IgG4 in most patients. Histological diagnosis is essential, and other key features include storiform fibrosis, lymphoplasmacytic infiltrate, tissue eosinophilia, and obliterative phlebitis. The disease can present with predominantly proliferative features, such as swollen lacrimal and salivary glands, orbital pseudotumor, autoimmune pancreatitis, polyclonal hypergammaglobulinemia (PHGG), eosinophilia, and tubulointerstitial nephritis of the kidneys, or predominantly fibrotic disease, including mediastinal and retroperitoneal fibrosis, sclerosing mesenteritis, and hypertrophic pachymeningitis. This review focuses on 4 key hematological manifestations: PHGG, IgG4-positive plasma cell enriched lymphadenopathy (LAD), eosinophilia, and retroperitoneal fibrosis (RPF). These features are found in 70%, 60%, 40%, and 25% of IgG4-RD patients, respectively, but can also represent key hematological “mimickers” of IgG4-RD, including Castleman disease (PHGG, LAD), eosinophilic vasculitis (eosinophilia, PHGG, LAD), hypereosinophilic syndromes (eosinophilia, LAD, PHGG), and histiocyte disorders (PHGG, LAD, RPF). An organized approach to these 4 manifestations, and how to distinguish IgG4-RD from its mimickers, is explained. Proliferative manifestations typically respond very well to treatment corticosteroids, rituximab, and other immunosuppressives, whereas chronic fibrotic disease may not be reversible with current treatment modalities.

01 Dec 2024·International Journal of Surgery Case Reports

Radical nephrectomy for retroperitoneal fibrosis: Case report

Article

Author: Stiirmer, Guilherme Henrique Silveira ; Moccellin, Eloi Guilherme Provinciali ; Dos Santos, Fernando Oliveira ; do Nascimento, Paulo Peixoto ; Vengjer, Alessandro ; Paiva, Matheus Miranda ; dos Santos, Fernando Oliveira

INTRODUCTION AND IMPORTANCERetroperitoneal fibrosis is a proliferative disease of fibroblasts with a still unclear appearance and low incidence. The clinical manifestations are nonspecific and appear late, pain is the most common symptom present. Elevated serum IgG4 levels are observed in up to 60 % of the patients and the main goal of treating this condition is to preserve kidney function.CASE PRESENTATIONWe present a case of an asymptomatic 34-year-old man. A poorly defined mass in the pre-aortic, pre-caval and right rim regions with possible malignancy was observed. After 3 biopsies, it was treated as low-grade follicular lymphoma with chemotherapy. With new growth after 1 year, right radical nephrectomy was performed to resect the lesion. Pathology showed that it was advanced retroperitoneal fibrosis with negative IgG4.CLINICAL DISCUSSIONRPF usually presents as an irregular mass of periaortic tissue that frequently has malignancy as a risk factor and may be associated with high levels of IgG4. Most of the time, the disease is asymptomatic. When the patient presents symptoms, pain is the most common, although late. Its diagnosis is made by imaging and histopathological exams. Treatment varies according to the progression of the disease, but aims to try to preserve renal function.CONCLUSIONRPF is a disease characterized by the accumulation of fibroblasts in the abdominal region with an etiology that has not yet been fully discovered, which can present in several ways, generally identified by imaging exams and can be treated individually depending on the invasiveness of the disease.

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