Sclerosing mesenteritis. Response to cyclophosphamide.
We present two patients with an aggressive form of sclerosing mesenteritis characterized by a progressive, life-threatening course, prominent retroperitoneal disease, and tubuloreticular structures in one case, an ultrastructural feature associated with autoimmune and cyclophosphamide-responsive diseases. In both patients, aggressive immunosuppressive medical therapy with cyclophosphamide resulted in prompt, dramatic improvement, without recurrence. When the diagnosis of sclerosing mesenteritis is established, we recommend early aggressive medical therapy with cyclophosphamide, particularly when tubuloreticular structures are present.
D000284:Administration, Oral; D000328:Adult; D003520:Cyclophosphamide / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010195:Pancreatitis / Q000188:drug therapy; D010201:Panniculitis, Nodular Nonsuppurative / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000503:physiopathology; D012186:Retroperitoneal Neoplasms / Q000473:pathology; D014057:Tomography, X-Ray Computed
D003520:Cyclophosphamide