Intestinal pseudo-obstruction in systemic lupus erythematosus: an uncommonbut important clinical manifestation

Objectives: To document intestinal pseudo-obstruction (IpsO) as a recognise d clinical manifestation of systemic lupus erythematosus (SLE) and a possib le new clinical entity with its apparent association with ureterohydronephr osis. Methodology: We report six lupus patients who presented with IpsO and revie w 12 other cases from an English literature search. IpsO is defined as the presence of clinical features suggestive of intestinal obstruction but with out organic obstruction, namely absence of bowel sounds, presence of multip le fluid levels on plain abdominal X-rays and exclusion of organic obstruct ion by imaging or surgical procedure. Other clinical characteristics relate d to the underlying lupus, serological and histological findings, treatment modalities and outcomes of these patients were reviewed. Results: All 18 patients fulfilled the ACR revised classification criteria for SLE. None showed any clinical features of scleroderma or overlap syndro me. The mean age of onset of IpsO was 29.0 (15-47) y. The female to male ra tio was 16 :2. Nine patients had IpsO as the initial presentation of their underlying lupus. Coexisting lupus involvement of other organ systems inclu ded glomerulonephritis (n=7), thrombocytopenia (n=5) and cerebral lupus (n= 3). The serology data and autoantibody profile of some of the previously re ported patients were incomplete. In our series, anti-Re antibody was positi ve in 5/6 while anti-RNP was found in 1/6 patients only. All our patients h ad active lupus serology at presentation. 17/18 patients required the use o f high dose systemic corticosteroid therapy while one patient responded to topical adrenocorticotrophin hormone treatment. Response was good and was o bserved early after commencement. Azathioprine was used as maintenance ther apy in 6/18 patients with good effects. An apparent association with the pr esence of bilateral ureterohydronephrosis was found in 12/18 patients. Thes e patients presented with dysuria without positive bacterial culture though features of chronic interstitial cystitis were not invariably found in the se patients. Conclusion: IpsO is an uncommon but important manifestation of SLE. The und erlying pathology is not fully understood but it may be related to immune c omplex deposition. The finding of coexisting ureterohydronephrosis suggests that there may also be a central smooth muscle motility problem of neuropa thic or myogenic pathophysiology which may or may not be secondary to vascu litis. Early recognition and treatment of IpsO in SLE is important.