A Prospective Study of Idiopathic Retroperitoneal Fibrosis in Adults Across China

What this Trial Is About

Researchers are investigating the disease progression and treatment responses of patients with idiopathic retroperitoneal fibrosis IRPF, a rare condition that causes inflammation and fibrosis around major abdominal arteries and can lead to kidney problems. This study focuses on Chinese patients diagnosed with IRPF to better understand their clinical features, demographics, laboratory and imaging results, and treatment outcomes. It is a prospective cohort study enrolling patients from across China. Patients with definite or possible IRPF will be included. Definite IRPF is identified by imaging showing retroperitoneal masses around the abdominal aorta and iliac arteries with ureteral involvement, no disease progression after glucocorticoid treatment, and specific histopathological signs of inflammation. Possible IRPF includes the imaging and treatment response criteria without biopsy confirmation. Patients with secondary forms of RPF or other autoimmune diseases are excluded. Data will be collected via an online platform, including symptoms, lab tests, imaging, pathology, treatment, and side effects. Participants will provide informed consent and contribute data over several years. Researchers will monitor organ involvement as the primary outcome over five years and assess treatment response, disease relapse, correlation between disease activity and relapse, survival rates, and imaging features during follow-up. Statistical analysis will summarize clinical and laboratory characteristics to improve understanding of IRPF and its management in the Chinese population.

A Prospective Cohort Study of IRPF in China