Idiopathic Retroperitoneal Fibrosis Clinical Trial
Official title:
A Prospective Cohort Study of Idiopathic Retroperitoneal Fibrosis in China
This is a cohort study to investigate the disease course and treatment response of patients
with idiopathic retroperitoneal fibrosis（IRPF）.
Methods: All the patients fulfilling diagnostic criteria of IRPF would be enrolled around
China. A online database system will 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.
Idiopathic retroperitoneal fibrosis（IRPF） is a rare fibro-inflammatory disease that develops
around the abdominal aorta and the iliac arteries, and spreads into the adjacent
retroperitoneum, where it frequently causes ureteral obstruction and renal failure. The
clinical phenotype of RPF is complex, because it can be associated with fibro-inflammatory
disorders involving other organs, and often arises in patients with other autoimmune
conditions.
This prospective cohort aims to study presentations of IRPF with laboratory findings and
imagings throughout the disease course. Newly diagnosed IRPF patients will be recruited from
all over the China.
Inclusion criteria: patients with definite, possible IRPF will be recruited.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: patients with malignancy or other autoimmune diseases are excluded.
Secondary forms of RPF related to drugs, infections, malignancies, Erdheim-Chester disease
and IgG4-RD, need to be excluded.
The data will be uploaded through the platform of Chinese Rheumatology Information Platform.
Demographic data, initial symptoms, disease duration, 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 including the demographic features,laboratory
characteristics, immunological tests, imaging and pathological features, in addition, the
treatment and prognosis of the disease.
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