Pneumatosis cystoides intestinalis in systemic diseases: 3 cases
Grasland, A.; Pouchot, J.; Leport, J.; Barge, J.; Vinceneux, P.
Presse Medicale 27(35): 1785-1788
1998
ISSN/ISBN: 0755-4982 PMID: 9850691 Document Number: 489586
Pneumatosis cystoides intestinalis (PCI) is usually a complication of digestive tract or respiratory tract diseases, but rare cases have been described in systemic diseases, mainly systemic sclerosis. Three patients, one with temporal arteritis and two with polyarteritis nodosa (complicating rheumatoid arthritis in one case) were treated by prednisone. All three developed PCI, complicated in one case by a retropneumoperitoneum. Medical treatment led to a favorable outcome in all cases. Sixty-two cases of PCI have been reported in patients with various systemic diseases (systemic sclerosis, systemic lupus erythematosus, mixed connective tissue disease, dermatopolymyositis, polyarteritis nodosa, rheumatoid arthritis, Sjögren's syndrome, amyloidosis). Systemic sclerosis is the most frequent condition (45%). In the other cases, corticosteroid therapy or digestive tract vasculitis are the main causal factors. Outcome is usually favorable with medical treatment. Laparotomy is rarely needed.