Pneumatosis cystoides intestinalis (PCI) is an uncommon disorder usually associated with intestinal and pulmonary obstructive diseases, recent abdominal procedures and systemic illnesses. PCI has been reported in patients with
systemic lupus erythematosus associated with intestinal
vasculitis. We describe herein a patient with a month history of intermittent
abdominal pain, diarrhoea, hyporexia, and
weight loss who underwent intestinal resection for
acute abdomen. Post-operatively she gave a three-month history of
arthritis of the right knee, ankles and feet,
arthralgia of the wrists, MCPs and shoulders. She also described weakness,
weight loss, Raynaud's phenomenon, and a
skin rash. Laboratory examination revealed an increased ESR, low haemoglobin and haematocrit, positive
rheumatoid factor, a positive ANA with a speckled pattern, as well
antibodies to
DNA, SS-A and
cardiolipin. The abdominal symptomatology especially
pain,
cramps and bouts of diarrhoea persisted after the surgery and became worse two months later. Abdominal X-ray showed distention of bowel with
cyst formation in the wall of the entire colon. A diagnosis of PCI was made radiologically. The intestinal pathology was reviewed and
vasculitis was identified. The patient received treatment with high dose
prednisone with an excellent response;
prednisone was progressively tapered and she has been asymptomatic without abdominal complaints or other symptoms for over a year.