To summarize the clinical features, treatment, and prognosis of ten children with childhood rheumatic diseases (CRDs) complicated with digestive tract (DT) perforation.

The clinical manifestations, laboratory tests, imaging characteristics, diagnosis, treatment, and prognosis of ten children with CRDs complicated with DT perforation admitted to the Children's Hospital of Capital Institute of Pediatrics from January 2013 to September 2021 were retrospectively analyzed.

The male-to-female ratio was 1∶1. There were seven cases of juvenile dermatomyositis (JDM), two cases of Behcet syndrome (BS) , and one case of systemic juvenile idiopathic arthritis (SJIA), accounting for 2.3% (7/302), 1.2% (2/165), and 0.4% (1/286) of the hospitalized children during the same period, respectively. The mean age of onset was (5.2±2.7) years (range, 8 months to 9.5 years). Seven children with JDM were strongly positive for antinuclear matrix protein 2 antibody (NXP2). The Childhood Myositis Assessment Scale (CMAS) score of six children with JDM was <5 points. Seven children with JDM and one child with BS received glucocorticoids and various immunosuppressants, but the primary disease continued to be unresolved. One child with BS and one with SJIA did not receive regular glucocorticoids and immunosuppressants. The median DT perforation time was 9 months (range, 1-34 months) after the diagnosis of the primary disease. The main clinical manifestation was unrelieved abdominal pain, and some children were accompanied by fever. All ten cases had peritonitis. After perforation, nine patients received high-dose methylprednisolone pulse therapy, eight received cyclophosphamide pulse therapy, and four (cases 2, 6, 8, and 9) received combined therapy with two or three immunosuppressants or biological agents. Seven children underwent routine DT perforation repair or ostomy. One patient (case 3) gave up treatment for economic reasons. Four cases survived, and the follow-up time was 6-48 months; six cases died.

The treatment efficacy of CRDs is unsatisfactory, and if persistent abdominal pain occurs, DT perforation should be suspected, especially in cases with NXP2-positive JDM. The mortality rate is high, and active treatment of the primary disease is the key, along with infection control, symptomatic surgical therapy, and nutritional support.