Intestinal Pseudo-Obstruction as a Presenting Manifestation of Systemic Lupus Erythematosus: Case Report and Review of the Literature

Hien Nguyen, MD; Niharika Khanna, MD

South Med J. 2004;97(2) 

In This Article

Discussion

Systemic lupus erythematosus is the most prevalent autoimmune disease, with an annual incidence of 60 per 1 million population and a prevalence of 500 per 1 million population.[1] Overall, systemic lupus erythematosus is most prevalent in the 20- to 40-year age group, with a female-to-male ratio of 9:1. Because of its myriad of multisystemic clinical presentations, systemic lupus erythematosus is known as one of the great medical mimics alongside tuberculosis, syphilis, and human immunodeficiency virus. I present a case of systemic lupus erythematosus in a patient whose initial presentation for the disease was renal failure and intestinal pseudo-obstruction. The patient met criteria for the diagnosis of systemic lupus erythematosus according to criteria of the American College of Rheumatology, including malar rash, arthritis, renal disorder with >0.5 g/dl/24 h protein, immunologic disorder with positive anti-Smith antibodies, false-positive Venereal Disease Research Laboratory test, positive antinuclear antibody, and hematologic disorder with Coombs-positive hemolytic anemia.[1] She did not meet criteria for other connective tissue diseases such as scleroderma, dermatopolymyositis, or overlap syndromes that have been reported in association with intestinal pseudo-obstruction.[1,2]

Gastrointestinal complications have been increasingly observed as manifestations of systemic lupus erythematosus. Up to 50% of patients with established systemic lupus erythematosus have gastrointestinal complaints at any time during the course of their illness.[1] These include mesenteric vasculitis, inflammatory bowel disease, pancreatitis, and less commonly motility disorders such as intestinal pseudo-obstruction.[1]

Intestinal pseudo-obstruction has been described in only 18 prior cases in association with systemic lupus erythematosus.[3,4] Intestinal pseudo-obstruction is defined as small bowel obstruction that occurs in the absence of mechanical or obstructive factors. Identification of systemic lupus erythematosus as the underlying pathologic process in this patient with small bowel obstruction was important because this facilitated steroidal therapy for reversal of the intestinal pseudo-obstruction and nephropathy and averted unnecessary surgical intervention.

This patient presented with classic symptoms of intestinal pseudo-obstruction, including abdominal pain and distension, vomiting, alternating diarrhea and constipation, weight loss, and radiographic signs of gaseous small bowel distension and air fluid levels.[3] Paradoxically, constipation and diarrhea may both be observed in patients with intestinal pseudo-obstruction because predominant small intestinal involvement may lead to bacterial overgrowth and steatorrhea, whereas predominant colonic involvement leads to constipation.[5,6] However, this all occurs in the absence of mechanical obstruction. Therefore, patients present with normal findings on gastroscopy, colonoscopy, and small bowel studies. Manometry studies in these patients demonstrate widespread gastrointestinal abnormalities, including small intestinal hypomotility; delayed gastric emptying; decreased lower esophageal sphincter pressure; and weakened contraction and migratory complexes in the stomach, antrum, duodenum, and jejunum.[3,5,6]

This patient's age and gender are consistent with the epidemiology of the 18 previous cases of systemic lupus erythematosus and intestinal pseudo-obstruction. In this group of 18 patients, there was a mean age of onset of 29 years (range, 15-47 yr) and a female-to-male ratio of 8:1. In one-half of these patients (9 of 18), intestinal pseudo-obstruction heralded the diagnosis of systemic lupus erythematosus.[3]

The pathophysiology of intestinal pseudo-obstruction is ineffective intestinal propulsion and intestinal hypomotility.[3,4] It is postulated that a generalized vasculitis involving inflammatory fibrinoid deposits affects the smooth muscle of the small intestine, leading to small bowel obstruction.[3,4,5] Smooth muscle dysfunction as a result of vasculitis may be more diffuse, as ureterovesical distension has been reported in patients with intestinal pseudo-obstruction. Mok and Wong[3] reported the coexistence of small bowel hypomotility and ureterohydronephrosis in 12 of 18 patients with intestinal pseudo-obstruction. Our patient's renal ultrasound demonstrated hydronephrosis, which is consistent with the possibility for coexistent urologic dysfunction. Thus, our patient's renal failure appears to be multifactorial from prerenal and intrinsic renal causes, including volume depletion from vomiting and diarrhea, mesangioproliferative changes in the glomerulus, and hydronephrosis arising from a possible vasculitis in the smooth muscles of the ureters and bladder.

The differential diagnosis of intestinal pseudo-obstruction in our patient who was eventually diagnosed with systemic lupus erythematosus included mechanical causes such as tumor, volvulus, and adhesions from her history of femoral hernia surgery; and nonmechanical causes such as gastroenteritis, paralytic ileus, celiac sprue, and narcotic bowel syndrome. Narcotic bowel was initially considered as a diagnosis for this patient who had a history of IV drug use, but drug screen was negative for illicit drugs. Narcotic bowel syndrome is a form of intestinal pseudo-obstruction characterized by chronic opiate use leading to ileus, vomiting, and abdominal pain.[7] The clinical manifestations of narcotic bowel can be remedied by clonidine during the period of narcotic withdrawal.[7] It is important to differentiate intestinal pseudo-obstruction arising from narcotic bowel versus that from systemic lupus erythematosus, because the treatment for the former is clonidine and treatment for the latter is steroids and promotility agents. Our patient was a diagnostic challenge because her history of femoral surgery and drug use led us to strongly consider alternate diagnoses such as surgical adhesions and narcotic bowel initially before the appropriate diagnosis of systemic lupus erythematosus.

Causes of intestinal pseudo-obstruction can be categorized into primary and secondary causes. Smooth muscle disorders, including familial visceral myopathy and sporadic visceral myopathy, are rare primary causes of intestinal pseudo-obstruction. Connective tissue disorders such as progressive systemic sclerosis, systemic lupus erythematosus, and dermatopolymyositis are secondary causes of intestinal pseudo-obstruction.[5] Other rare secondary causes of intestinal pseudo-obstruction include progressive muscular dystrophy, amyloidosis, diffuse lymphoid infiltration, Parkinson's disease, myxedema, pheochromocytoma, and opiate drugs.[5]

Therapy for intestinal pseudo-obstruction consists of bowel rest with parenteral nutrition, and high-dose IV steroids. Solu-Medrol (Pharmacia & Upjohn, Kalamazoo, MI) is initiated at 1 mg/kg/d for 3 days, followed by oral prednisone 1 mg/kg/d.[3,4] In addition, pharmacologic stimulation of gut motility can be achieved with octreotide or erythromycin.[3] Octreotide is a somatostatin analogue that is administered subcutaneously to induce migratory motor complexes in the small intestine.[8] One previous patient with systemic lupus erythematosus and intestinal pseudo-obstruction was treated successfully with adrenocorticotropic hormone.[3] In our case, this patient was successfully treated with a combination of IV steroids and erythromycin, with clinical remission observed within 1 week of therapy. Long-term outcome with intestinal pseudo-obstruction in patients with systemic lupus erythematosus is not well defined. In one small series of six patients, small bowel motility was restored to normal between 1 and 8 weeks after onset of symptoms. In these patients, prednisone was continued up to 2 months' duration, and all were in remission at 48 months of follow-up.[3] Azathioprine was used for maintenance therapy in 6 of the 18 previously reported cases of patients with systemic lupus erythematosus and intestinal pseudo-obstruction.[3] This suggests that intestinal pseudo-obstruction secondary to systemic lupus erythematosus may require chronic immunosuppression for sustained remission. Our patient has been in remission after 1 year without the necessity for chronic steroids or other immunosuppressants.

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