Table 1.
The clinical features of 18 patients with PCI.
| Case | Age (years), sex | Disease duration (months) | Disease activity | Past history | GI tract presentation before the onset of PCI | Symptoms at PCI onset | PCI region | Free air in the abdominal cavity | PCI treatment | Prognosis | Pathological |
|---|---|---|---|---|---|---|---|---|---|---|---|
| P1/1993 (11) | ND, F | ND | Stable | ILD | ND | ND | ND | Yes | ND | ND | None a |
| P2/1999 (12) | 61, F | 17 | Stable | No | Hiatal hernia + atrophic gastritis | Abdominal pain + abdominal distension | Ileum | Yes | Surgery | Remission | None b |
| P3/2004 (13) (Mi-2) |
58, F | 8 | Active | No | Dysphagia | No | Ascending colon | Yes | Fasting | Remission | ND |
| P4/2005 (14) | 69, F | 120 | Stable | No | No | Abdominal distension | Jejunum, ileum | Yes | Oxygen therapy | Remission | Nonec,d |
| P5/2006 (15) | 53, F | 48 | Active | Diabetes (voglibose) | Dysphagia | Abdominal distension | Ascending colon, descending colon | Yes | Termination of AGI | Remission | ND |
| P6/2008 (16) | 53, F | 3 | Active | No | Constipation | Abdominal pain | Jejunum | Yes | Oxygen therapy, bowel rest, antibiotics | Remission | ND |
| P7/2008 (17) | 30, F | 84 | Stable | No | Dysphagia + constipation | Abdominal distension | Small intestine, ascending colon | Yes | Fasting | Remission | ND |
| p8/2012 (18) | 67, F | 12 | Stable | No | No | Diarrhea | Colon | Yes | Oxygen therapy, fasting, antibiotics | Remission | ND |
| P9/2012 (7) | 59, F | 36 | Stable | ILD | No | Abdominal distension | Colon | Yes | Antibiotics | Remission | ND |
| P10/2012 (7) | 41, F | 240 | Active | ILD | No | Abdominal pain | Ileum | Yes | Surgery | Death/ARDS | None e |
| P11/2013 (19) | 51, F | 32 | Active | No | No | Nausea + vomiting + fever | Transverse colon | Yes | Surgery, oxygen therapy, fasting, antibiotics | Remission | None f |
| P12/2017 (20) | 70, M | ND | Active | No | Dysphagia | Abdominal distension | Ascending colon | Yes | Oxygen therapy, fasting, antibiotics | Death/infections | Noneg,h |
| P13 SAE |
60, F | 10 | Relapse | Diabetes (acarbose) | Dysphagia | No | Ascending colon | Yes | No | Remission | ND |
| P14 TIF1r |
61, M | 7 | Relapse | Mediastinal emphysema Diabetes (acarbose, sitagliptin) |
Dysphagia | No | Ascending colon | Yes | No | – | ND |
| P15 MDA5 |
49, M | 10 | Stable | Diabetes (acarbose) | No | No | Ascending colon | Yes | No | Remission | ND |
| P16 MDA5 |
69, F | 4 | Stable | Diabetes (insulin) Skin infection |
No | Temporary abdominal pain | Ascending colon | Yes | No | – | ND |
| P17 MDA5 |
59, F | 2 | Stable | Mediastinal emphysema Diabetes (acarbose, sitagliptin) |
No | No | Ascending colon | Yes | Fasting and antibiotics for 1 day | Remission | ND |
| P18 MDA5 |
57, F | 7 | Stable | Diabetes (acarbose, metformin, linagliptin) Endoscopy |
Constipation | No | Ascending colon | No | No | Remission | ND |
AGI, alpha-glucosidase inhibitor; None, no vasculitis; ND, no data.
Ischemic changes were seen but biopsy specimens did not show vasculitis.
The resected bowel showed typical histological features of pneumatosis cystoides intestinalis with a giant cell reaction around the gas-filled cysts. No evidence of vasculitis or intravascular thrombosis.
Colonoscopy was performed to examine the colon and ileum. Although the colon and rectum were intact, polypoid lesions like submucosal tumors (SMTs) with redness of the mucosa were seen in the ileum (approximately 30 cm beyond the ileum end). The polyps were elastic and movable, and biopsy specimens showed non-specific inflammation with no air-filled cysts.
Endoscopic pathological.
Both macro- and microscopic findings revealed that the muscularis propria was disrupted and replaced with fat tissue. There was no pathological finding of abscess or vasculitis.
Colon histopathology. Cystic spaces within the submucosa and subserosa are focally lined by histiocytes, a classic feature of pneumatosis intestinalis. Submucosal cystic dilation typical of pneumatosis intestinalis is present. Lymphoid aggregate is normal in appearance. PCI was found unrelated to infection, malignancy, or vasculitis, and no frank perforation was present.
No abnormalities, such as gas or fistula, were evident in the small or large intestines on histological examination at autopsy.
Autopsy pathological.
"-", No follow-up result.