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Table 1 Clinical data of JDM patients with GI perforation

From: Gastrointestinal perforation in anti-NXP2 antibody-associated juvenile dermatomyositis: case reports and a review of the literature

Patient
/Year
Gender Age at onset (y) CMAS score CAT-A
score
CAT-D
score
Positive for MSA Treatment before
GI perforation
Symptoms of GI perforation Duration from onset to perforation (m) Site of perforation Surgical
treatment
Histopathology of perforation site Medical Treatment Follow-up(m)
/Outcome
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
  1. F is female, M is male, ND is no data, y is year, CMAS is child myositis assessment score, MSA is myositis-specific antibody, m is month, Pred is prednisone, CYC is cyclophosphamide, IVIG is intravenous immunoglobulin, MTX is methotrexate, MP is methylprednisol, CsA is cyclosporine A, RTX is rituximab, AZA is azathioprine, Thal is thalidomide, HCQ is hydroxychloroquine, Po is peros, CMV is Cytomegalovirus, PE is plasma exchanges, ARDS is acute respiratory distresssyndrome, CAT-A is cutaneous assessment tool activity, CAT-D is cutaneous assessment tool damage