Patients with collagen diseases have been reported to demonstrate a greater risk when undergoing surgical operations. To determine the risk factors in abdominal surgery for patients with collagen diseases, 32 patients with collagen diseases who underwent abdominal surgery were analyzed for their clinical features and surgical results by comparing 26 cases from the favorable prognosis group (Group A) and 6 cases resulting in hospital death (Group B). The analysis revealed that emergent operations tended to result in worse outcomes (P = 0.011) than elective operations and that cases undergoing operations for collagen disease-related problems, including intestinal perforation and acute pancreatitis, also showed a worse postoperative course than those who underwent operations for problems unrelated to collagen diseases, such as carcinomas and cholelithiasis (P = 0.0006). The dose of steroids administered at the time of operation was also significantly higher in Group B than in Group A (P = 0.03). These results suggested that the patients with collagen diseases should be followed periodically not only for the primary disease but also for any potential surgical diseases to identify such diseases at an early stage and to avoid an emergent operation, and that patients treated with high doses of steroids also need intensive care after abdominal surgery.