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

doi:10.21203/rs.3.rs-34258/v1

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

https://doi.org/10.21203/rs.3.rs-34258/v1

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published 06 Jan, 2021

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Background: To summarize the characteristics of gastrointestinal (GI) perforation in anti-NXP2 antibody-associated juvenile dermatomyositis (JDM).

Methods: Five patients with GI perforation from a JDM cohort of 120 cases are described. Relevant literature was reviewed.

Results: Five patients, including 4 females and 1 male, were involved in the study. The average age of the 5 patients was 5.74 years (5.74 ±2.82). Myositis-specific antibody (MAS) spectrum analysis indicated that the five patients were anti-NXP2 antibody positive. The initial symptoms of GI perforation were progressive abdominal pain and intermittent fever. Two patients also presented with ureteral calculus with hydronephrosis and ureteral stricture. Surgery was performed in four patients, which was successful in only one patient through fistulization. Four patients died (4/5, 80%), and 1 patient was completely relieved after 2 years of treatment. Combined with 16 patients described in the literature, the onset symptom was progressive abdominal pain, which often occurred within 10 months after JDM was diagnosed. Perforation most often occurred in the duodenum, although perforations at multiple sites or recurrent perforations also occurred. The mortality rate of GI perforation in JDM was 38% (8/21).

Conclusions: All perforation cases subjected to MAS analysis were anti-NXP2 antibody positive. The onset symptom was abdominal pain. The most common site of perforation was the duodenum in the retroperitoneum, and the lack of acute abdominal manifestations complicated early diagnosis. GI perforation is a fatal complication in JDM, and early diagnosis is very important. More research is needed to determine the pathogenesis and predictive factors of GI perforation in JDM.

Rheumatology

juvenile dermatomyositis

gastrointestinal perforation

anti-NXP2 antibody.

Juvenile dermatomyositis (JDM) is an autoimmune disease characterized by proximal myopathy and a characteristic rash. However, multiple systems, including the digestive system, can also be involved. The occurrence of gastrointestinal (GI) perforation is rare, but a high mortality rate has been reported due to atypical symptoms and difficulty in early diagnosis[1]. Myositis-specific autoantibodies (MSAs) are increasingly used to delineate distinct subgroups of JDM[2, 3]. The presence of anti-NXP2 autoantibodies substantially increases the risk of calcinosis across all ages and is associated with disease severity[4]. The performance of MSAs in cases of JDM complicated by GI perforation has not been reported. Here, from among 120 patients with JDM, we report five cases complicated by GI perforation. All 5 patients had anti-NXP2 antibody-associated JDM. In addition, we reviewed JDM cases with GI perforation reported in the literature to improve our recognition of this disease. Anti-NXP2 autoantibodies may facilitate early diagnosis of the disease.

We describe five patients with GI perforation from a JDM cohort who were admitted to the Children’s Hospital affiliated with the Capital Institute of Pediatrics, China, from January 2016 to December 2019. The study passed a review by the Ethics Committee of Capital Institute of Pediatrics.. All patients met the diagnostic criteria for definite dermatomyositis[5]. Sixteen DM-specific antibodies (MDA-5, NXP2, Jo-1, PM-SCL100, PL-7, PL-12, EJ, OJ, anti-Ro52, Ku, PM-Scl, SRP, SAE1, TIF1γ, Mi-2α and Mi-2β) were tested by immune dot blot hybridization using a commercial kit based on goat anti-human IgG antibodies (BlueDot Myositis 12 IgG, #MYO12D-24, D-TEK, Belgium). We searched PubMed, Medline and Scopus using the keywords “juvenile dermatomyositis”, “gastrointestinal perforation”, and “perforation”. Articles with detailed cases were reviewed.

The general data of the 5 patients

Five patients with GI perforation were identified from 120 cases in a JDM cohort (5/120, 4.17%). Eighty percent of JDM patients complicated by GI perforation were female (4/5, 80%), and the average age was 5.74 years (5.74 ±2.82). All 5 patients showed severe rashes, skin ulcers, and severe weakness of the skeletal muscles (from 3/5 to 1/5). No calcification was observed. Analysis of the sixteen DM-specific antibodies showed that 5 patients were anti-NXP2 antibody positive. The copy number of cytomegalovirus (CMV)-DNA was increased in the blood of one patient. GI perforation occurred from 4 to 13 months after JDM was diagnosed. The onset symptoms were severe abdominal pain and fever, GI bleeding and vomiting. Perforations occurred in the duodenum (3/5, 75%), duodenum and colon (1/5, 25%), and an unclear location (1/5, 25%), and perforations at multiple sites or recurrent perforations occurred in 4 patients (4/5, 80%). All patients were treated with corticosteroids and immunosuppressant agents (cyclophosphamide (CTX)/methotrexate (MTX)/cyclosporine A (CsA)). One patient was treated with plasma exchange (PE) therapy, and two patients were treated with rituximab (RTX). Surgery was performed in 4 patients, which was successful in only one patient through fistulization. Four patients died (4/5, 80%), and the symptoms of one patient were completely relieved after 24 months of follow-up.

Case 1 A 3.4-year-old girl suffering from rash and muscle weakness was diagnosed with JDM in a local hospital and was treated with prednisone(Pred 2 mg/kg.d) and MTX (12 mg/m².w). Muscle weakness was relieved after 3 months of treatment, and the dosage of Pred was reduced. When the Pred was reduced to 10 mg/d, infection in the lungs, ulceration around the anus and abdominal pain developed. One month later, a perforation in the transverse colon was confirmed, and colostomy was performed in a local hospital. After she was treated with Pred, CTX, intravenous immunoglobulin (IVIG) and MTX, her abdominal pain disappeared, and her muscle strength improved. Seven months after JDM diagnosis, ureteral calculus with hydronephrosis and ureteral stricture were diagnosed and treated via ureteroscopy. Nine months after JDM diagnosis, severe abdominal pain and more severe muscle weakness developed, and pulse MP and CsA were given. Later, GI bleeding and fever occurred again. She was transferred to the emergency department. Shock was confirmed, abdominal contrast-enhanced computed tomography (CT) indicated encapsulated effusion and pneumatosis, and duodenal perforation was confirmed during surgery. After duodenostomy, Pred, pulse CTX and thalidomide were given, and then we switched to oral Pred, CTX and MTX after her condition stabilized. Two years later, her symptoms were completely relieved, and ureteral stricture disappeared.

Case 2 A 5-year-old girl suffering from rash and muscle weakness was diagnosed with JDM in a local hospital and treated with Pre, MTX and hydroxychloroquine (HCQ), and then her muscle strength improved. Her parents stopped the treatment. Twelve months after JDM diagnosis, she was admitted to our emergency department due to severe malnutrition, skin rash, muscle weakness, abdominal pain, vomiting and dark green stool. Contrast-enhanced CT indicated peritonitis, intestinal perforation and a right renal-ureteral calculus with pelvicalyceal-ureteral dilation. During surgery, multiple perforations of the duodenum were identified, and the right hepatic artery was ruptured. The ruptured blood vessel was sutured, and duodenal jejunal tube placement was unsuccessful. The patient died of infectious and haemorrhagic shock.

Case 3 A 3.3-year-old boy suffering from a skin rash and muscle weakness was diagnosed with JDM and treated with Pred, MTX and IVIG in a local hospital. His symptoms improved after treatment; the dosage of Pred was reduced, and MTX was stopped due to cataracts and abnormal liver function. However, physical examination revealed a scattered rash and crusted ulcers all over the body and severe proximal muscle weakness. MP, RTX and IVIG were administered in our hospital. Two weeks later, he gradually developed abdominal pain, bloating, low back pain, right lower quadrant tenderness and rebound pain. Localized colitis was suspected based on a CT scan. His abdominal pain and distension were relieved after the administration of antibiotics, MP (20 mg/kg.d×2 d) and IVIG. Four days later, he developed fever with abdominal pain and distension, and an ultrasound showed echo enhancement in the omentum of the right lower quadrant with weak peristalsis and pelvic effusion. MP, pulse CTX and IVIG were continued, and his temperature returned to normal with improved abdominal pain. However, 7 days later, he experienced severe abdominal pain and distension with a rapid decrease in his haemoglobin (HB) level (from 112 g/L to 67 g/L). Abdominal muscle inflammation and haemorrhage were indicated on ultrasound, and contrast-enhanced CT showed generalized retroperitoneal infection with abdominal wall haemorrhage and intestinal perforation (Fig. 1). Surgical intervention was not possible. Repeated blood transfusion, anti-infection therapy, and treatment for the primary disease were given. He still had abdominal pain and bloating. A slight change in body position caused massive abdominal wall bleeding. He died after returning home.

Case 4 A 7-year-old girl with a past history of JDM was treated 1 year earlier with MP and an immunosuppressant. She presented with progressive proximal muscle weakness and skin ulcers. She presented with intermittent abdominal pain for 1 month. Physical examination showed severe malnutrition, anaemia, a scattered rash and ulcers all over the body, proximal muscle weakness and oedema in both upper limbs. Her HB level was 59 g/L. Abdominal contrast-enhanced CT did not indicate obvious abnormalities. Ultrasound of the abdomen suggested thickening of the abdominal bowel wall and poor bowel movements. After blood transfusion treatment, MP and CTX were given. Her symptoms improved, and abdominal pain disappeared. HB decreased again after 2 weeks, and fever and abdominal pain appeared again 4 weeks later. Abdominal CT revealed bowel perforation. She immediately underwent surgical repair (Fig. 2). One week later, abdominal pain appeared again. Abdominal CT showed another intestinal perforation. Surgical repair and PE therapy were performed. Her abdominal pain was relieved, but the pulmonary infection was still severe. Three days later, she had peritonitis manifestations and then suffered from convulsions. Pulse MP and anti-infection agents were given. Surgical intervention was not possible. The patient died due to uncontrolled bleeding and refractory shock.

Case 5 A 10-year-old girl had been diagnosed with JDM 6 months earlier and received Pred and immunosuppressant treatment. Intermittent abdominal pain and fever for 1 month were reported. Initial treatment included pulse MP and IVIG. Muscle weakness and rash did not improve. She was admitted to our hospital with GI haemorrhage for 3 days. Physical examination showed a poor mental response, painful expression, severe malnutrition, anaemia, a scattered rash and ulcers throughout the body (Fig. 3), and severe proximal muscle weakness. Her HB level was 54 g/L. Ultrasound of the abdomen suggested thickening of the abdominal bowel wall and poor bowel movements. Pulse RTX and MP were given. Three days later, her abdominal pain worsened. Ultrasound of the abdomen showed intestinal perforation. During surgery, a 5-cm perforation of the horizontal section of the duodenum was observed, and a fistula was repaired. Her abdominal pain was relieved. CMV-DNA was detected in the blood, and she was given ganciclovir treatment. Four days later, she experienced abdominal pain and muscle tension again. Considering the necrosis and perforation of the lower part of the duodenum, her parents withdrew treatment, and the patient then died.

Results of the literature review

Sixteen patients with JDM with GI perforation in twelve studies[6-18] were identified in PubMed, Medline and Scopus. The studies were published between 1984 and 2020. Eight patients were female (8/16, 50%), six patients were male (6/16, 37.5%), and the sexes of two patients were not recorded (2/16, 12.5%). The average age was 6.51 years (6.51± 2.33). No clear MSA results were recorded in any case. Primary treatment with Pred and immunosuppressants did not have a significant effect. GI symptoms occurred from 1 to 108 months after treatment, most of which occurred within 10 months. The first GI symptoms were severe abdominal pain with fever or vomiting. Perforations were reported in the duodenum (10/16, 62.5%), colon (6/16 patients, 37.5%), jejunum (2/16, 12.5%), oesophagus (2/16, 12.5%), gastric pylorus (1/16, 6.25%), caecum (1/16, 6.25%) and an unclear location (1/16, 6.25%); perforations at multiple sites or recurrent perforations were reported in six patients (6/16, 37.5%). Only 5 patients had records regarding treatment of the initial disease, and they were treated with Pred/MP and an immunosuppressant (CTX/CsA). One patient underwent PE therapy, and one patient received PE therapy and RTX. Surgery was performed in 14 patients. In particular, one patient was treated with ganciclovir. Four patients died (4/16, 25%), and the others were discharged from the hospital and experienced relief of their symptoms. The medical information of all sixteen patients from the literature and the five patients in our hospital is summarized in Table 1.

JDM is an autoimmune disease with chronic nonsuppurative inflammation chiefly involving the skeletal muscles and skin, the pathology of which is generalized vasculitis [19, 20]. Multiple systems can be involved, but GI perforations in anti-NXP2 antibody-associated JDM are rarely reported. Most patients in our study were female, with a proportion that was significantly higher than that for the patients reported in the literature. The average age of the five patients in our study was 5.74 years (5.74 ±2.82), and GI involvement appeared between 4 and 13 months postdiagnosis, which was similar to the 16 cases reported in the literature. The initial symptoms of JDM with GI perforation were severe abdominal pain, abdominal distention, vomiting, fever and bloody stools. Early identification of perforations was very difficult. With regard to P1 and P2, the doctors did not recognize that GI perforation had occurred after persistent abdominal pain for more than 1 month. The perforation in P3 occurred during hospitalization in our hospital and manifested as severe abdominal pain and distension with decreased bowel sounds. Repeated abdominal ultrasounds, X-rays and contrast-enhanced CTs indicated the presence of a low-signal mass in the right lower quadrant with bowel distension, thinning of the intestinal wall and local colitis without pneumoperitoneum or subphrenic pneumatosis. Transitory relief of symptoms occurred after treatment, but severe abdominal pain quickly reappeared, and contrast-enhanced CT and ultrasound showed intestinal perforation, diffuse abdominal wall bleeding and infection. We did not know the precise time when the perforation occurred, suggesting that once abdominal pain appears, perforation should be strongly suspected, and imaging should be performed. Perforation should be considered when abdominal pain is not relieved by active treatment in patients with JDM, and perforation can be diagnosed early with contrast-enhanced CT scans[6]. We also suggest that repeated scanning and GI angiograms are essential if the diagnosis cannot be confirmed during the first scan. In the 16 patients in the literature, perforation often occurred in the duodenum (partially in the retroperitoneal area in P6) and the colon. In addition, multisite or recurrent perforations were also observed in some patients (P7,P10,P16,P18,P20,P21), similar to the results of our study. Furthermore, arterial rupture near the perforation site often occurs, as observed in P2, who had ruptured hepatic arteries, and in P10, who had a ruptured duodenal proximal mesenteric artery; therefore, haemorrhage should also be considered when an unexplained decrease in the HB level occurs in JDM patients.

In addition, 2 patients (P1,P2) had urinary system involvement in this group of cases, including ureteral stones, ureteropelvic dilation and ureteral stenosis. With remission of the primary disease, ureteral stricture disappeared in P1, and no ureteral stones were found. These findings may be related to vasculitis involving the urinary system[21]. Ruby Haviv[22] reported a case of JDM with right ureteral obstruction secondary to necrosis. The histopathology report of the resected ureter revealed moderate, acute, and chronic inflammatory infiltrates within the ureter wall, urothelial erosions, superficial granulation tissue, degenerated smooth muscle fibres within the muscularis propria, some of which were infiltrated with lymphocytes and plasma cells, and multiple calcifications within the lamina propria. Fragments of gross calcifications were also noted. Similar calcifications were observed in other parts of the ureter. Obstructing calcifications were also noted within small blood vessels. Thus, abdominal vasculopathy in JDM may lead to ureteral stones and ureteral stricture as in other forms of vasculitis.

The underlying mechanisms of GI perforation in JDM are still not clear. Some researchers have reported glucocorticoids, NSAIDs and MTX to be risk factors for GI ulceration and perforation, especially at one month post-treatment or with an accumulated dose of steroids higher than 1 g[17]. In our study, Pred was reduced to a small dose before GI symptoms appeared in P1. After perforation was identified, pulse MP pulse and CTX were given, and the patient’s condition improved; thus, the perforation was not attributed to drug factors. Since the pathology of JDM is vasculitis, the basic pathology of ulcers and perforations may also be vasculitis. Schneider KO et al[6] assumed that ulceration in the proximal region and bulb of the duodenum was related to medication, while perforation in the descending part was related to vasculitis, causing intestinal ischaemia and necrosis due to thrombogenesis and vascular occlusion. Recent researchers reported that perforated sites showed chronic vasculopathy rather than acute vasculitis[16,17]. This chronic vasculopathy is characterized by narrowing or complete occlusion of multiple small and medium arteries, subintimal foam cells, fibromyxoid neointimal expansion, and significant luminal compromise with infiltration of macrophages through the muscle layers into the intima. Histopathology at the perforation site was recorded in 3 patients (P6, P12, P19) in the literature[6,9,13], and no vasculitis was noted. P12 exhibited mucosal ischaemia, and P19 exhibited vasculopathy and CMV colitis. No manifestation of vasculitis was observed in the pathology of the fistula. Therefore, whether chronic noninflammatory vasculopathy causes ischaemia and perforation must be considered. Bhaskaran H et al[13] reported a case of intestinal perforation in a child with JDM caused by CMV colitis. CMV-DNA was also detected in the blood of P5. Because she was not aware of the possibility of CMV colitis, no relevant pathological tests were performed. The other 4 patients did not undergo blood CMV-DNA tests. The role of CMV colitis in perforation requires further study.

The one hundred twenty patients in the JDM cohort were all tested by MAS analysis, all patients with GI perforation had strong positivity for anti-NXP2 antibody (3+), and no GI perforation was noted in other MSA patients, which led us question whether anti-NXP2 antibody is associated with GI perforation. After reviewing 16 cases in the literature, Besnard C. et al[13] reported that most patients with severe GI manifestations were positive for anti-NXP2 antibody or anti-TIF-1γ antibody. Unfortunately, no specific antibody type was recorded in 2 patients with GI perforation in this study. The other patients described in the literature had not been tested for MSA. In 2017, Tansley SL et al[23] mentioned that 14 children who were positive for anti-NXP2 antibody had ulcers. Aouizerate J et al[24] reported that anti-NXP2 antibody was positively associated with GI involvement in JDM. The study evaluated the clinical, biological, and histological manifestations of 23 JDM patients and performed a multivariate analysis of 26 histopathological parameters. Anti-NXP2 antibody-positive patients were reported to have more GI manifestations. A case-control study can be performed to further uncover the relationship between anti-NXP2 antibody and GI perforation, which may further reveal the pathological mechanism of GI perforation. Based on our experience in this study, if the primary disease in anti-NXP2 antibody-positive patients with JDM does not resolve for a long time, clinicians should be highly alert to the possibility of GI perforation when abdominal pain occurs.

The treatment and prognosis of patients with JDM and GI perforation were also assessed in our study. The mortality rate among our patients (4/5, 80%) was higher than that reported in the literature (4/16, 25%), which might be due to their severe conditions and poor responses to various therapeutic drugs, delayed surgery and operational difficulty at the perforation site. When a patient has intermittent abdominal pain and GI ulcers, the primary disease should be treated more actively to prevent GI perforation. In addition to glucocorticoids and immunosuppressants, RTX and PE therapy can be used as treatment options[25.26]. Once GI perforation occurs, the primary disease is not well controlled, and the prognosis is poor.

In this study, the clinical details of 5 cases of anti-NXP2 antibody-associated JDM complicated by GI perforation were summarized for the first time. Based on the cases combined with a literature review, once JDM patients present with abdominal pain, especially anti-NXP2 antibody-positive patients, GI perforation should be considered. For timely diagnosis, dynamic imaging is important. GI perforation is a fatal complication of JDM, and more research is needed.

JDM	juvenile dermatomyositis
NXP2	nuclear matrix protein 2
GI	Gastrointestinal
CMAS	child myositis assessment score
Pred	prednisone
CTX	cyclophosphamide
IVIG	intravenous immunoglobulin
MTX	methotrexate
MP	methylprednisol
CsA	cyclosporine A
RTX	rituximab
AZA	azathioprine
Thal	thalidomide
Po	peros
MRS	methicillin-resistant staphylococcus
PE	plasma exchanges
MSAs	Myositis-specific autoantibodies
CMV	Cytomegalovirus
CT	computed tomography
HCQ	hydroxychloroquine
HB	haemoglobin
MRI	magnetic resonance imaging
y	year
m	month
ND	no data
F	female
M	male
ARDS	acute respiratory distresssyndrome

Ethics approval and consent to participation

Parental informed consent for publication was obtained. This study was approved by the Ethics Committee of Capital Institute of Pediatrics.

Consent for publication

Written informed consent was obtained from the patient’s legal guardians for publication of this case report and any accompanying images.

Competing interests

The authors declare that they have no competing interests.

Funding

This work was supported by Beijing Municipal Administration of Hospitals Clinical Medicine Development of Special Funding Support (code:XMLX201813) and Capital Funds for Health Improvement and Research (code:2020-2-2102).

Authors’ contributions

YX wrote the first draft of the manuscript, contributed to patient management and to the literature review. XM made many contributions in the process of writing and revising the manuscript. ZZ supervised patient management. JL critically revised the manuscript and supervised patient management. JH,JZ,MK and JL contributed to patient management. XL revised the manuscript. All the authors read and approved the final version of the manuscript.

Acknowledgement

We would like to thank the Department of General Surgery, the Department of Intensive Medicine and the Department of Radiology of the Children's Hospital Affiliated to the Capital Institute of Pediatrics for their contributions to the treatment of patients in our study.

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Table 1 Clinical data of JDM patients with GI perforation

Patient

/Year

Gender

Age (y)

CMAS 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

3.4

Anti-NXP2

Pred+MTX+IVIG

Abdominal pain

bloody stools

4/9

Duodenum; colon

Transverse colon ostomy;

duodenostomy

Inflammatory cells infiltrate;

No vasculitis

Pred+Mp pulse+CsA+

IVIG→Pred+Thal+CTX→

Pred+CTX (Po)+MTX

24/Remission

5.0

Anti-NXP2

MP+MP Pulse+CTX

HCQ/MP+MP Pulse+MTX

Abdominal pain

bloody stools

Duodenum;

Duodenal bulb hepatic

artery rupture

Vessel sutured;

placement of duodenal

jejunal tube (failed)

Pred+MP Pulse+CTX+

IVIG

Death/Infection

3.3

Anti-NXP2

Pred+MTX+IVIG/

Pred+IVIG +RTX

Abdominal pain

and distention bloody stools

Unknown

No surgery

MP pulse+CTX +IVIG

Death/Abdominal bleeding

Anti-NXP2

Pred+ MTX/CsA

→MP pulse+IVIG+CTX

Abdominal pain

bloody stools

Duodenum×2

Perforation repair×2

CTX→MP pulse+IVIG+PE

Death/Shock

Anti-NXP2

Pred+ MTX/CsA

Abdominal pain 、fever and

bloody stools

Duodenum×2

Perforation repair

MP pulse+ RTX

Death/Abandon treatment

P6^[6]

/2016

7.0

Pred+MTX

Abdominal pain vomiting and fever

Duodenal retroperitoneum

Perforation repair

No vasculitis

ND/Improved

P7^[7]

/1997

7.0

Pred

Abdominal pain vomit

Duodenum Jejunum

Perforation repair

Pred+MP pulse+CTX

12/Stable

P8^[8]

/1988

5.0

Pred

Abdominal pain

Colon

Perforation repair

12/Stable

P9^[8]

/1988

7.0

Pred +MTX

Abdominal pain

The third duodenum

3/ND

P10^[8]

/1988

8.0

Pred+MTX

Abdominal pain

fever

Esophagus;

duodenum; colon

Mesenteric arterial rupture

Perforation repair

ND/Improved

P11^[8]

/1988

6.0

Pred+MTX

Abdominal pain and distention/fever

Duodenum

Perforation repair

Death/unknown

P12^[9]

/2001

4.0

Pred+AZA

Abdominal pain/

vomiting/fever

Duodenum

Perforation repair

Mucosal ischemia

Pred+MP pulse

19/ND

P13^[10]

/2014

2.0

Abdominal pain

108

Unknown

Death/unknown

P14^[11]

/1985

4.0

Gastric pylorus

Partial gastrectomy and gastrojejunostomy

ND/Stable.

P15^[11]

/1985

5.0

Middle transverse colon

Loop transverse colon fistula

Death/ARDS

P16^[11]

/1985

6.0

Pred+MTX/Pred+AZA/

Pre+CTX

Abdominal pain/

vomiting/fever

Duodenum colon transverse colon

Multiple perforation repair

7/Stable.

P17^[11]

/1985

9.0

Pred/Pred+MTX/

Pred+AZA

Abdominal pain/ fever

Duodenum

Perforation repair

ND/Stable.

P18 ^[12]

/1984

7.0

Pred+MTX

Abdominal pain

Duodenal perforation ,

colon perforations

Perforation repair

48/Stable.

P19 ^[13]

/2019

6.0

Pred+MTX

Abdominal pain/fever

transverse colon,

Perforation repair

and loop colostomy.

No vasculitis and vasculopathy,

CMV colitis

Steroid+IVIG+Ganciclovir

12/Remission

P20^[14]

/2020

10.5

Abdominal pain, GI hemorrhage, severe bowel obstruction

8/20

Duodenal Perforation,

Jejunal perforation

Jejunal resection, exclusive parenteral

nutrition, antibiotics,

Steroid+CsA

108/Remission

P21^[14]

/2020

10.7

Abdominal pain,

severe bowel obstruction

7/9

Esophageal perforation

Cecal perforation

Colostomy

Steroid+ RTX+ PE

84/Death/Hepatitis

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published 06 Jan, 2021

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

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