A Prospective Study of Cyclophosphamide Treatment for Idiopathic Retroperitoneal Fibrosis
A Prospective Study of Cyclophosphamide Treatment for Idiopathic Retroperitoneal Fibrosis
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.
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.
Inclusion Criteria:

 -  age 18-75 diagnosed as IRPF. The diagnosis of idiopathic retroperitoneal fibrosis is
 based on the following aspects :(1) the swelling of retroperitoneal tissue , which
 seems neoplastic; (2) A large number of lymphocytes proliferated and infiltrated in
 the affected tissues and organs, and tissues showed inflammation, fibrosis and
 sclerosis, in which IgG4-positive cells accounted for less than 50% of plasma cells;
 (3) Increased inflammatory markers, such as ESR and CRP; (4) Good response to
 glucocorticoid therapy.

 Exclusion Criteria:

 -  malignancy retroperitoneal fibrosis secondary to other diseases or drugs or abdominal
 surgeries.