Abstract:

Objective To investigate the clinical characteristics, diagnosis and treatment of children with juvenile dermatomyositis (JDM) combined with severe gastrointestinal involvement. Methods Clinical data, diagnosis and treatment of three children with JDM combined with severe gastrointestinal involvement and gastrointestinal perforation were retrospectively analyzed, and a literature review was conducted. Results All three cases were girls, with onset of disease between 2 years 7 months and 8 years of age. Gastrointestinal manifestations were observed within a timeframe ranging from 2 to 26 months subsequent to the diagnosis of juvenile dermatomyositis. In the first case, the individual presented with abdominal pain, abdominal distension, fever, vomiting, and hematochezia, accompanied by reduced bowel sounds. In the second case, the individual experienced gastrointestinal symptoms characterized by temporary abdominal pain that resolved without intervention. There were no apparent gastrointestinal complaints seen in case 3. The first example exhibited a duodenal bulb ulcer characterized by the presence of exposed blood vessels, along with concurrent conditions of oesophagitis, appendicitis, and pancreatitis. Asymptomatic gastrointestinal perforation was observed in cases 2 and 3. Hemostatic treatment was administered to case 1, which included fasting, gastrointestinal decompression, methylprednisolone, cyclosporine, meropenem, and a growth inhibitor. The patient experienced an amelioration of gastrointestinal complaints, however, there was a subsequent decline in muscle strength. Subsequently, the patient was discharged in an automated manner and subsequently succumbed to mortality. Cases 2 and 3 were subjected to fasting, laxative administration, methylprednisolone treatment, and cyclophosphamide shock therapy. In addition, case 2 received supportive treatment with human immunoglobulin, while two children underwent pneumoperitoneum. In conclusion, it can be inferred that the aforementioned points support the notion that the user’s gastrointestinal perforation has the potential to show at any point throughout the progression of juvenile dermatomyositis (JDM) in children. However, the initial clinical signs of this complication are often inconspicuous and challenging to identify. Once overt gastrointestinal symptoms become apparent, the illness reaches a critical state and the prognosis tends to be unfavorable. Following the occurrence of gastrointestinal perforation, it becomes imperative to initiate aggressive therapy for the underlying condition and proceed with surgicalintervention.

Key words: juvenile dermatomyositis, gastrointestinal perforation, child