Clinical trial NCT04314323: National Registry of IRPF in China
Official title: National Registry of Idiopathic Retroperitoneal Fibrosis in China
Study type: OBSERVATIONAL
Overall status: RECRUITING
Conditions: Idiopathic Retroperitoneal Fibrosis
Keywords: Registry; Cohort-study; Idiopathic retroperitoneal fibrosis；
Lead sponsor: Peking Union Medical College Hospital
Sponsor class: OTHER
First posted: 2020-03-19
Enrollment: 500
Sex: ALL
Minimum age (years): 18
Maximum age (years): 75
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: IRPF patients around China.

Brief summary: 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.

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.

Eligibility criteria: 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 outcomes:
measure: Organ involvements of Chinese IRPF patients
timeFrame: 5 years
Secondary outcomes:
measure: Response rate of treatment with glucocorticoids and immunosuppressants on IRPF in China
timeFrame: 5 years
measure: Relapse rate of IRPF patients in China
timeFrame: 5 years
measure: The correlation between baseline disease activities and relapse rate.
timeFrame: 5 years
measure: Ten year survival rate of patients with IRPF in China.
timeFrame: 10 years
measure: The imaging features of involved organs.
timeFrame: 3 years
Other outcomes:
Locations:
city: Beijing
country: China
facility: Peking Union Medical College Hospital
state: Beijing
Study officials:
References: