Chronic intestinal pseudo-obstruction in systemic lupus erythematosus due to intestinal smooth muscle myopathy

We report the case of a woman with systemic lupus erythematosus initially m anifesting with fever, rash and arthritis, and two years later with Class I V lupus nephritis. Following treatment with cyclophosphamide she developed symptoms and signs of chronic intestinal pseudo-obstruction (CIPO) that was initially thought to be due to a neutropenic enterocolitis. However, persi stence of symptoms resulted in segmental resection of the ileum which showe d widespread myocyte necrosis and active inflammation within the muscularis propria. A subsequent, more extensive ileocolic resection showed severe di ffuse atrophy and fibrosis of the muscularis propria throughout the resecte d bowel. The absence of mesenteric vasculitis and the clinical response of the CIPO to the immunosupressive regimen of prednisolone and cyclosporin A suggest that the bowel muscle coat changes reflect an intestinal myopathy s econdary to systemic lupus erythematosus, and may have an auto-immune etiol ogy.