National Registry of IRPF in China

National Registry of Idiopathic Retroperitoneal Fibrosis in China

The aim of this study is to establish a nation-wide cohort study of idiopathic retroperitoneal fibrosis (IRPF) in China.

Methods: All the patients fulfilling diagnostic criteria of IRPF would be enrolled from multi-centers around China. A online database system has been established.

Endpoints: The primary endpoint is to investigate the clinical manifestations of Chinese IRPF patients; the secondary endpoints including the demographic features,laboratory characteristics, immunological tests, imaging and pathological features, in addition, the treatment and prognosis of the disease.

Study Overview

• Status: Recruiting
• Conditions: Idiopathic Retroperitoneal Fibrosis

Detailed Description

This is a multicenter registry study of IRPF patients. Newly diagnosed IRPF patients will be recruited from all over the China. Inclusion criteria: patients with newly diagnosed IRPF will be recruited. Exclusion criteria: patients with secondary forms of retroperitoneal fibrosis or malignancy are excluded.

The data will be uploaded through the platform of Chinese Rheumatology Information Platform. Demographic data, initial symptoms, disease duration, history of allergy, and physical examination, organ involvements, laboratory findings, radiological and pathological results, as well as treatment, side effects will be recorded.

This study was approved by the Medical Ethics Committee of PUMCH (Beijing, China). All patients will sign informed consent.

Statistical Analysis: All parameters are described in the standard summary statistics, including mean, standard deviation, minimum, and maximum. All statistical analyses will be performed by SPSS.

Endpoints: The primary endpoint is to investigate the organ involvements of Chinese IRPF patients. The secondary endpoints include the demographic features,laboratory characteristics, immunological tests, imaging and pathological features, in addition, the treatment and prognosis of the disease.

• Study Type: Observational
• Enrollment (Anticipated): 500

Contacts and Locations

Study Locations

• China
  • Beijing
    • Beijing, Beijing, China, 100032
      • Recruiting
      • Peking Union Medical College Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 75 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

IRPF patients around China.

Description

Inclusion Criteria:

• Conforming to the diagnostic criteria of IRPF;Definite IRPF must have the following characteristics: (1) The typical finding of Imaging shows retroperitoneal mass, surrounding the abdominal aorta and iliac arteries, with encasement of ureters and/or organ dysfunction; (2) Exclusion of disease progression after standard glucocorticoid treatment; and (3) histopathological findings shows a fibrous tissue with signs of chronic inflammation. The inflammatory component is comprised of lymphocytes, plasma cells and macrophages. Neutrophils and granulomas are rare findings. Possible IRPF required (1) and (2), but without histopathological examination.

Exclusion Criteria:

• None of them met the criteria for other autoimmune diseases. None had malignant disease. Secondary forms of RPF related to drugs, infections, malignancies, Erdheim-Chester disease and IgG4-RD, need to be excluded.

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Organ involvements of Chinese IRPF patients	To calculate the percentage of organ involvements in at least 500 patients.	5 years

Secondary Outcome Measures

Outcome Measure	Time Frame
The correlation between baseline disease activities and relapse rate.	5 years
The imaging features of involved organs.	3 years
Response rate of treatment with glucocorticoids and immunosuppressants on IRPF in China	5 years
Relapse rate of IRPF patients in China	5 years
Ten year survival rate of patients with IRPF in China.	10 years

Collaborators and Investigators

• Sponsor: Peking Union Medical College Hospital

Study record dates

Study Major Dates

• Study Start (Anticipated): March 17, 2020
• Primary Completion (Anticipated): March 1, 2030
• Study Completion (Anticipated): March 1, 2030

Study Registration Dates

• First Submitted: March 17, 2020
• First Submitted That Met QC Criteria: March 17, 2020
• First Posted (Actual): March 19, 2020

Study Record Updates

• Last Update Posted (Actual): March 19, 2020
• Last Update Submitted That Met QC Criteria: March 17, 2020
• Last Verified: March 1, 2020

More Information

Terms related to this study

• Keywords: Registry; Cohort-study; Idiopathic retroperitoneal fibrosis；
• Additional Relevant MeSH Terms: Pathologic Processes; Fibrosis; Retroperitoneal Fibrosis
• Other Study ID Numbers: IRPF group in China

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: Yes