Case report
Gastrointestinal vasculitis and pneumatosis intestinalis due to systemic lupus erythematosus: successful treatment with pulse intravenous cyclophosphamide

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  • Cited by (74)

    • Benign pneumatosis intestinalis in a pediatric patient with multiple risk factors including granulomatosis with polyangiitis: A case report and review of the literature

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      However, symptoms do not always correlate with severity of PI. Similar to our patient, 6% of previously reported patients with PI and rheumatologic disease were asymptomatic [2–73]. PI typically affects the jejunum and the ileum, with only 6% of cases affecting the colon, but in this cohort of patients, 42% of patients had involvement of the small bowel, 28% had involvement of the large bowel, and 10% had involvement of both [1–73,77].

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      Patients intolerant of steroids or having disease recurrence following tapering off of steroids can be given pulsed doses of cyclophosphamide as an alternative. This alkylating antineoplastic and immunosuppressant agent has been used in four cases, with successful results in three patients.2,8-10 In severe cases, surgical removal of the bowel has been reported.11

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