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Severe juvenile dermatomyositis complicated by pancreatitis
1Department of Rheumatology, Great Ormond Street Hospital for Sick Children, London.
Abstract:
We report two boys with juvenile dermatomyositis (JDM) complicated by pancreatitis. One also had hepatitis and probably mild bowel vasculitis, while the other had catastrophic bowel vasculitis with multiple perforations. Both were on corticosteroids, but had features of active vasculitis. The former improved with high-dose i.v. pulsed methylprednisolone, while the latter improved only after immunosuppression with i.v. methylprednisolone, cyclophosphamide and plasmapheresis. Although bowel vasculitis is a known complication of severe JDM, pancreatitis and hepatitis are extremely rare. We have found in a literature search only three other reports of pancreatitis complicating JDM. We wish to alert physicians that pancreatitis may develop in JDM. It should be considered as a differential diagnosis in the child with active disease who develops abdominal pain. Control of vasculitis with adequate immunosuppression, as well as general supportive measures, may be valuable in the treatment of pancreatitis in JDM.
Insights
Pancreatitis is a rare but serious complication of juvenile dermatomyositis (JDM). Physicians should consider pancreatitis in JDM patients with abdominal pain, especially those with active vasculitis.
Area of Science:
- Pediatrics
- Rheumatology
- Gastroenterology
Background:
- Juvenile dermatomyositis (JDM) is a rare autoimmune disease affecting children.
- While gastrointestinal complications like bowel vasculitis are known, pancreatitis is exceptionally rare in JDM.
Observation:
- This report details two boys with JDM who developed pancreatitis.
- One patient experienced hepatitis and mild bowel vasculitis; the other had severe bowel vasculitis with perforations.
- Both patients had active vasculitis despite corticosteroid treatment.
Findings:
- The first patient improved with high-dose intravenous pulsed methylprednisolone.
- The second patient required intensive immunosuppression, including intravenous methylprednisolone, cyclophosphamide, and plasmapheresis, for improvement.
- Literature search revealed only three prior reports of pancreatitis in JDM.
Implications:
- Pancreatitis should be considered in the differential diagnosis of abdominal pain in children with active JDM.
- Effective management of pancreatitis in JDM may involve controlling the underlying vasculitis with immunosuppression and supportive care.