Table 1 Clinical data of JDM patients with GI perforation

P1

F

3.4

5

2

3

Anti-NXP2

Pred+MTX + IVIG

Abdominal pain/

bloody stools

4/9

Duodenum/ colon

Transverse colon ostomyv

duodenostomy

Inflammatory cells infiltrate/

No vasculitis

Pred+Mp pulse+CsA+

IVIG→Pred+Thal+CYC→

Pred+CYC (Po) + MTX → Pred+MTX

24/Remission

P2

F

5.0

1

7

5

Anti-NXP2

MP + MP Pulse+CYC

HCQ/MP + MP Pulse+MTX

Abdominal pain/

bloody stools

10

Duodenum/

Duodenal bulb hepatic

artery rupture

Vessel sutured/

placement of duodenal

jejunal tube (failed)

ND

Pred+MP Pulse+CYC+

IVIG

Death/Infection

P3

M

3.3

5

6

5

Anti-NXP2

Pred+MTX + IVIG/

Pred+IVIG +RTX

Abdominal pain

and distention bloody stools

10

Unknown

No surgery

ND

MP pulse+CYC + IVIG

Death/Abdominal bleeding

P4

F

6

2

6

6

Anti-NXP2

Pred+ MTX/CsA

→MP pulse+IVIG+CYC

Abdominal pain/

bloody stools

13

Duodenum×2

Perforation repair×2

ND

CYC → MP pulse +IVIG+PE

Death/Shock

P5

F

9.5

2

6

4

Anti-NXP2

Pred+ MTX/CsA

Abdominal pain/ fever and bloody stools

6

Duodenum×2

Perforation repair

ND

MP pulse+ RTX

Death/Abandon treatment

P6 [6]

/2016

F

7.0

ND

ND

ND

ND

Pred+MTX

Abdominal pain vomiting and fever

6

Duodenal retroperitoneum

Perforation repair

No vasculitis

ND

ND/Improved

P7 [7]

/1997

M

7.0

ND

ND

ND

ND

Pred

Abdominal pain /vomit

24

Duodenum Jejunum

Perforation repair

ND

PE

Pred+MP pulse+CYC

12/Stable

P8 [8]

/1988

F

5.0

ND

ND

ND

ND

Pred

Abdominal pain

3

Colon

Perforation repair

ND

ND

12/Stable

P9 [8]

/1988

F

7.0

ND

ND

ND

ND

Pred +MTX

Abdominal pain

6

The third duodenum

ND

ND

ND

3/ND

P10 [8]

/1988

M

8.0

ND

ND

ND

ND

Pred+MTX

Abdominal pain/

fever

6

Esophagus/

duodenum/ colon

Mesenteric arterial rupture

Perforation repair

ND

ND

ND/Improved

P11 [8]

/1988

F

6.0

ND

ND

ND

ND

Pred+MTX

Abdominal pain and distention/fever

5

Duodenum

Perforation repair

ND

ND

Death/unknown

P12 [9]

/2001

M

4.0

ND

ND

ND

ND

Pred+AZA

Abdominal pain/

vomiting/fever

2

Duodenum

Perforation repair

Mucosal ischemia

Pred+MP pulse

19/ND

P13 [10]

/2014

M

2.0

ND

ND

ND

ND

ND

Abdominal pain

108

Unknown

ND

ND

ND

Death/unknown

P14 [11]

/1985

F

4.0

ND

ND

ND

ND

ND

ND

48

Gastric pylorus

Partial gastrectomy and gastrojejunostomy

ND

ND

ND/Stable.

P15 [11]

/1985

F

5.0

ND

ND

ND

ND

ND

ND

3

Middle transverse colon

Loop transverse colon fistula

ND

 ND

Death/ARDS

P16 [11]

/1985

F

6.0

ND

ND

ND

ND

Pred+MTX/Pred+AZA/

Pre + CYC

Abdominal pain/

vomiting/fever

10

Duodenum colon transverse colon

Multiple perforation repair

ND

ND

7/Stable.

P17 [11]

/1985

M

9.0

ND

ND

ND

ND

Pred/Pred+MTX/

Pred+AZA

Abdominal pain/ fever

13

Duodenum

Perforation repair

ND

ND

ND/Stable.

P18 [12]

/1984

M

7.0

ND

ND

ND

ND

Pred+MTX

Abdominal pain

10

Duodenal perforation/

colon perforations

Perforation repair

ND

ND

48/Stable.

P19 [13]

/2019

F

6.0

ND

ND

ND

ND

Pred+MTX

Abdominal pain/fever

1

transverse colon,

Perforation repair

and loop colostomy.

No vasculitis and vasculopathy/

CMV colitis

Steroid+IVIG+Ganciclovir

12/Remission

P20 [14]

/2020

ND

10.5

16

ND

ND

ND

ND

Abdominal pain/ GI hemorrhage/ severe bowel obstruction

8/20

Duodenal Perforation/

Jejunal perforation

Jejunal resection/ exclusive parenteral

nutrition/ antibiotics,

ND

Steroid+CsA

108/Remission

P21 [14]

/2020

ND

10.7

5

ND

ND

ND

ND

Abdominal pain/

severe bowel obstruction

7/9

Esophageal perforation/

Cecal perforation

Colostomy

ND

Steroid+ RTX+ PE

84/Death/Hepatitis