Idiopathic retroperitoneal fibrosis is a chronic disease that requires treatment for a few years. Patients with this disease respond well to glucocorticoid therapy, so you need to take glucocorticoid medication once the diagnosis is established. In addition, most patients need to use immunosuppressants when taking glucocorticoids. At present, the most commonly used immunosuppressants in the world are cyclophosphamide, azathioprine, methotrexate and mycophoranate, etc. The function of the above drugs is to improve the disorder of the immune system function, in order to restore the normal immune state, so that the inflammation and swelling of the affected organs disappear, restore the normal function. It should be emphasized that if the use of hormones and immunosuppressant is not standard or stopped too early, it can lead to disease recurrence. Therefore, after the disease is under control, long-term adherence to medication is needed to maintain a stable state of the disease.~At present, there is no clear treatment guideline for idiopathic retroperitoneal fibrosis in the world, and the addition of immunosuppressant is also out of debate. Therefore, a comprehensive evaluation of its pathogenesis, clinical characteristics and treatment response is needed.
This prospective, interventional, controlled study is aimed to evaluate the efficacy and safety of cyclophosphamide in the treatment of idiopathic retroperitoneal fibrosis, which refers to the chronic nonspecific inflammation of retroperitoneal fascia and adipose tissue that gradually evolves into fibroproliferative disease.