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Intestinal pseudo-obstruction as an initial presentation of systemic sclerosis in two children
O Ortiz-Alvarez1, D Cabral, J S Prendiville
1Department of Pediatrics, University of British, Columbia Vancouver, Canada.
Insights
Systemic sclerosis initially presented as intestinal pseudo-obstruction in two children. Treatments led to symptom improvement, though gastrointestinal radiological changes persisted.
Area of Science:
- Pediatrics
- Gastroenterology
- Rheumatology
Background:
- Systemic sclerosis is a rare autoimmune disease affecting connective tissues.
- Gastrointestinal involvement is common in systemic sclerosis, but initial presentation as intestinal pseudo-obstruction in children is infrequent.
Observation:
- Two pediatric cases of systemic sclerosis are presented.
- Intestinal pseudo-obstruction was the primary presenting symptom in both children.
Findings:
- Both patients showed improvement in gastrointestinal symptoms and skin manifestations after treatment.
- Case 1 received prednisone and penicillamine; Case 2 was treated with methotrexate.
- Radiological evidence of gastrointestinal changes persisted for 3 years (Case 1) and 2 years (Case 2) post-treatment.
Implications:
- Early diagnosis and treatment of systemic sclerosis can lead to symptom resolution in pediatric patients.
- Intestinal pseudo-obstruction should be considered in the differential diagnosis of pediatric gastrointestinal issues.
- Long-term monitoring for persistent radiological changes is warranted even after clinical improvement.
Abstract:
Two children are reported in whom intestinal pseudo-obstruction was the initial manifestation of systemic sclerosis. Gastrointestinal symptoms and skin changes resolved or improved in both children following treatment with prednisone and penicillamine (case 1) or methotrexate (case 2), although radiological changes of the gastrointestinal tract persisted at 3 and 2 yr of follow-up, respectively.