Remmele W, Müller-Lobeck H, Paulus W
Institut für Pathologie, Dr. Horst-Schmidt-Kliniken, Wiesbaden, FRG.
Pathol Res Pract. 1988 Dec;184(1):77-85. doi: 10.1016/S0344-0338(88)80194-8.
Primary mesenteritis is a rare disease. Two cases and two additional patients with mesenteric fibrosis/fibromatosis are reported. A classification of primary and secondary mesenteritis is suggested in order to replace the confusing variety of terms used for the same disease process. Differential diagnosis of mesenteric fibrosis versus mesenteric fibromatosis may be difficult, and some criteria for the separation of these two entities are discussed. Among the clinical symptoms, transmission of aortic pulsations to the anterior abdominal wall is a rare but important aid for diagnosis. Associated diseases are malignant lymphoma, colonic adenomatosis (Gardner's syndrome) and retroperitoneal fibrosis. Microscopically, mesenteric lipomatosis and Whipple's disease must be ruled out. Usually, surgical treatment is not necessary. Prednisone and azathioprine may be helpful in severe cases. Fatal outcome of primary mesenteritis is extremely rare.
原发性肠系膜炎是一种罕见疾病。本文报告了2例原发性肠系膜炎以及另外2例肠系膜纤维化/纤维瘤病患者。为了取代用于同一疾病过程的各种令人困惑的术语，建议对原发性和继发性肠系膜炎进行分类。肠系膜纤维化与肠系膜纤维瘤病的鉴别诊断可能困难，本文讨论了区分这两种实体的一些标准。在临床症状中，主动脉搏动传至前腹壁虽罕见但对诊断有重要帮助。相关疾病有恶性淋巴瘤、结肠腺瘤病（加德纳综合征）和腹膜后纤维化。在显微镜下，必须排除肠系膜脂肪过多症和惠普尔病。通常，无需手术治疗。在严重病例中，泼尼松和硫唑嘌呤可能有用。原发性肠系膜炎的致命结局极为罕见。
