Four Cases of Pneumatosis Cystoides Intestinalis Complicated by Connective Tissue Diseases

Abstract

Pneumatosis cystoides intestinalis (PCI) is a rare disease that involves the presence of gas in the intestinal wall. Connective tissue disease (CTD) is a major cause of secondary PCI. In addition to the nature of CTDs, the use of prednisolone and some immunosuppressants, and the presence of complicating diseases such as diabetes mellitus, constipation and pulmonary diseases are involved in the development of PCI. This report describes four cases of PCI with different CTDs (granulomatosis with polyangiitis, rheumatoid arthritis, dermatomyositis, and overlap syndrome) and discusses the background of each patient and common risk factors for the occurrence of PCI.

Pneumatosis Intestinalis Developed in a Patient with Giant Cell Arteritis While in a Clinically Sustained Remission Phase

“…Thus, our case is considered the first case of PI in GCA with no other cause. A variety of underlying etiologies have been proposed to explain the abnormal accumulation of gas in PI; however, several theories have been advanced (1,6,12), including (1) the mechanical theory, in which increased intestinal pressure due to intestinal obstruction results in a break in the integrity of the gastric mucosa and serves as the driving force in PI; (2) the pulmonary theory, in which pulmonary alveolar rupture caused by pulmonary diseases, such as chronic obstructive disease or interstitial pneumonitis, produce gas dissecting interstitially along the bronchopulmonary bundles to the mediastinum and retroperitoneally along the aorta and the mesenteric vessels to the bowel wall; (3) the bacterial theory, in which the gas is produced by gas-forming bacteria that enter the mucosal barrier or increased mucosal permeability and produce gas within the bowel wall; and (4) the chemical or nutritional deficiency theory, in which malnutrition increases bacterial fermentation in the intestine, thus producing large volumes of gas and subsequently the submucosal dissection of gas. Recently, the development of PI during treatment with a-glucosidase inhibitors (a-GIs) has been reported (8,13,14).…”

“…We herein report a case of PI complicated by GCA. There have been some reports on PI with CTDs ( 6 - 9 ). The major primary diseases associated with PI among CTDs are SSc ( 3 , 4 ) and polymyositis/dermatomyositis ( 6 ), followed by SLE ( 5 , 7 , 10 ) and mixed connective tissue disease ( 9 ).…”

“…There have been some reports on PI with CTDs ( 6 - 9 ). The major primary diseases associated with PI among CTDs are SSc ( 3 , 4 ) and polymyositis/dermatomyositis ( 6 ), followed by SLE ( 5 , 7 , 10 ) and mixed connective tissue disease ( 9 ). After we experienced this case, we searched the English literature though PubMed using the key search terms ‘pneumatosis intestinalis’, ‘pneumoperitoneum’, and ‘giant cell arteritis’.…”

“…Among the various causes of PI, connective tissue diseases (CTDs) are infrequent ( 2 ). Nevertheless, PI has been reported in patients with systemic sclerosis (SSc) ( 3 , 4 ), systemic lupus erythematosus (SLE) ( 5 ), and other CTDs ( 6 , 7 ). However, no report has yet described a case of PI presenting in a patient with giant cell arteritis (GCA), one of the most common vasculitides affecting large vessels in elderly patients, causing a variety of symptoms.…”

An asymptomatic case of pneumatosis cystoids intestinalis: A case report

“…PCI can be divided into primary (15%) and secondary (85%) types [5]. Various gastrointestinal and non-gastrointestinal diseases has been considered as causes of secondary types of PCI, such as bowel ischemia [6], inflammatory bowel diseases, bowel infection, gastroduodenal ulcer, necrotizing enterocolitis [5], pyloric stenosis [7], diverticular disease [5], obstructive pulmonary disease [8], drugs (e.g., alpha-glucosidase inhibitors) [9,10], collagen tissue diseases [11,12], acquired immune deficiency syndrome [13] and malnutrition [4,5,14]. Anywhere none of these symptoms and diseases affected our patient, so his type of PCI can be cosidered as primary type.…”