|
| | Onset age of PI | Period from JDM onset to PI onset | Treatment before PI onset | Manifestations of PI | Image findings and clinical diagnosis | Disease activity of JDM at PI onset | Clinical course of PI | Outcome |
|
| 1 [13] | 4.8, female | 1 Y | GCs | Abdominal pain, diarrhea, constipation, and movable mass in the left lower quadrant | Cystoid gas collection in the mid transverse colon and splenic flexure | Calcinosis universalis was noted | Pneumatosis persisted for 25 months | Survived |
|
| 2 [14] | 8.5, female | 3 Y | GCs, AZP, CY, and MTX | Abdominal pain and abdominal distention | Gas-filled hepatic flexure of colon and extraluminal gas | Refractory myositis and skin manifestations with disseminated subcutaneous calcification | Abdominal manifestations remitted during the next 10 days with PSL and MTX. Intramural gas persisted for four months | Survived |
|
| 3 [15] | 12, female | 3 Y | None | Dysphagia | Cystoid gas collection | Noted myositis and dermatitis | Treated with oral GCs | Survived |
|
| 4 [10] | 8.5, female | 1 Mo | GCs and MTX | Abdominal pain, vomiting, diarrhea, and fever | Perforation and peritonitis | Increased muscle weakness, rash, and rising muscle enzyme values recurred | Died 6 weeks after the development of PI owing to complications of perforations, peritonitis, and candida sepsis | Died |
|
| 5 [11] | 11, female | 3 Mo | GCs and CY | Abdominal pain, bilious emesis, and fever | Extensive extraluminal gas collection in the right abdomen and flank.
Peritonitis, retroperitoneal abscess, and duodenal perforation were found at laparotomy | Became bedridden because of progressive muscle weakness from JDM onset | Discharged 8.5 months after admission over multiple episodes of sepsis and 8 laparotomies | Survived |
|
| 6 [11] | 15, male | 2 Y | GCs | Abdominal discomfort, pain, vomiting, fever, and hematemesis | Following appendicitis and appendectomy, PI occurred with duodenal perforation and peritonitis | Exacerbated muscle weakness two months previously | Died of perforation, sepsis and multiple organ failure on the 21st hospital day despite four laparotomies | Died |
|
| 7 [12] | 7, female | 3 Mo | GCs and MTX | Abdominal pain | Intramural air in the ascending and transverse colon | Prominent skin rash and vascular ulcers in the axillar, minimal proximal muscle weakness | Treated with intravenous antibiotics and parental nutrition. Clinical improvement evident after a week | Survived |
|
| 8 [8] | 8, female | 1 Mo | GCs, MTX, and mPSL pulse | Cough and abdominal distention | CT showed extensive PI in the large colon | Increased weakness and a vasculitic ulcer on upper eyelid and in the nare | Treated with intravenous antibiotics and a short period of bowel rest | Survived |
|
| Present case | 16, female | 6 Y | GCs | Abdominal pain and abdominal distention | Extensive gas-filled colon and extraluminal gas above colon | Intermittently worsened skin manifestations | Complicated with strangulated obstruction and large intestine resection and colostomy were performed | Survived |
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