Recurrent pneumatosis intestinalis in a patient with dermatomyositis

Abstract

SUMMARYA 51-year-old woman with dermatomyositis (DM) on chronic immunosuppressive therapy was hospitalised for evaluation of haematuria. Surprisingly, abdominal imaging demonstrated pneumoperitoneum and pneumatosis intestinalis (PI). Her abdominal examination and white cell count were normal, but she subsequently developed nausea and fever. Owing to concern for perforation, a hemicolectomy was performed. Pathology revealed PI without inflammatory, ischaemic or neoplastic features, and she recovered uneventfully. Her immunosuppressive therapy was discontinued. Six months later, a follow-up CT of the abdomen revealed recurrence of PI. As she was asymptomatic, she was managed conservatively with resolution of PI on subsequent imaging. PI is characterised by the presence of gas within the wall of the intestine. Its aetiology is often unclear but this case highlights the association between PI and both immunosuppressive therapy and DM. A review of PI in patients with DM suggests that clinically stable patients may be observed, while avoiding surgical intervention. BACKGROUND

Pneumatosis Intestinalis in Lung Cancer Induced Twice by Different Drugs: Bevacizumab and Pemetrexed

“…It is reported to manifest as gas found in a linear or cystic form in the submucosa or subserosa of the bowel wall ( 1 ). Pneumatosis intestinalis is associated with various conditions, such as pulmonary disease ( 3 , 4 ), gastrointestinal disease ( 4 ), collagen disease ( 4 - 6 ), infectious disease ( 7 , 8 ), and iatrogenic disorder ( 9 ). Moreover, pneumatosis intestinalis is found in association with the use of certain drugs, including steroids ( 4 , 5 , 10 , 11 ) and α-glucosidase inhibitors ( 5 , 12 ).…”

Successful open abdomen treatment for multiple ischemic duodenal perforated ulcers in dermatomyositis

“…Many cases involve the duodenum and particularly the third portion and its retroperitoneal aspect [ 3 - 5 , 9 , 11 , 16 , 17 , 19 ]. Other typical sites of perforation are the esophagus [ 6 , 14 - 16 ], the cecum, and the right and left colon in their retroperitoneal portion [ 2 , 7 - 10 , 13 ]. Histopathological findings are related to acute arteriopathy, with arterial and venous intimal hyperplasia and occlusion of vessels by fibrin thrombi.…”

Barium‐induced pneumatosis intestinalis in a patient with anti‐synthetase syndrome

“…PI is diagnosed based on the presence of intramucosal gas, which can develop as a rare complication of connective tissue diseases. 1 , 2 Microangiopathy and steroid treatment have been proposed as its pathogenesis in patients with connective tissue diseases. 1 , 2 Also, increased intestinal pressure by intestinal obstruction is assumed to trigger the PI.…”

“… 1 , 2 Microangiopathy and steroid treatment have been proposed as its pathogenesis in patients with connective tissue diseases. 1 , 2 Also, increased intestinal pressure by intestinal obstruction is assumed to trigger the PI. 3 In our case, intestinal obstruction due to impaction of barium contrast medium may have triggered PI in association with mucosal fragility due to anti‐synthetase syndrome and steroid treatment.…”