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.
Eligibility
Sex
ALL
Min age
18 Years
Max age
75 Years
Healthy volunteers
No
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.
Primary outcome measure(s)
Organ involvements of Chinese IRPF patients — 5 years To calculate the percentage of organ involvements in at least 500 patients.
Trial sites (1)
Facility
City
Region
Status
Peking Union Medical College Hospital
Beijing
Beijing Municipality
Recruiting
More Peking Union Medical College Hospital trials in China
This page summarises publicly available registry data for informational purposes — not medical advice. Eligibility is determined by each study team; patients should discuss participation with their clinician.
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