Elsevier

Human Immunology

Volume 76, Issue 9, September 2015, Pages 695-700
Human Immunology

Review
A review of 42 cases of intestinal pseudo-obstruction in patients with systemic lupus erythematosus based on case reports

https://doi.org/10.1016/j.humimm.2015.09.022Get rights and content

Abstract

Intestinal pseudo-obstruction (IpsO) is considered a severe manifestation of systemic lupus erythematosus (SLE) characterized by clinical and radiological evidence of intestinal obstruction with no identifiable mechanical lesion. We performed a systematic review to document IpsO in SLE. Twenty-eight articles with 42 patients were included. The median age of onset of IpsO was 27.5 (10–57) years. The female to male ratio was 38:4. Twenty-two (52.4%) patients had IpsO as the initial presentation of their underlying lupus. Three (7.1%) patients manifested in inactive lupus. The duration of abdominal symptoms before admitted ranged from 3 days to 3 years, however most of the patients responded well to systemic corticosteroid or immunosuppressive treatment within 2 days to about 3 months. Concomitant ureterohydronephrosis was present in approximately three-fourths of the cases. More interestingly, 4 patients presented hepatobiliary dilatation without mechanical obstruction together with IPO and ureterohydronephrosis. In conclusion, IpsO is an uncommon but important manifestation of SLE. The finding of coexisting ureterohydronephrosis and hepatobiliary dilatation suggests that there may be generalized visceral muscle dysmotility. Early recognition of IpsO is necessary to institute appropriate medical treatment and to avoid inappropriate surgical intervention.

Introduction

SLE is a multisystemic autoimmune inflammatory disease with a variety of presenting features and manifestations. SLE can involve any part of the gastrointestinal tract with oral lesions, esophageal dysmotility, mesenteric vasculitis, protein-losing enteropathy and pancreatitis the most frequent manifestations [1], [2]. Gastrointestinal symptoms are common in patients with SLE, with more than half of them complaining of anorexia, nausea and vomiting [1]. The symptoms may be directly attributable to SLE, intercurrent illness or side-effects of medication [1]. Abdominal complaints in patients with SLE may pose a difficult diagnostic challenge with the possible differential diagnoses of primary gastrointestinal disorders, manifestations of SLE on the gastrointestinal tract and side effects of therapy for SLE. IpsO is a rare clinical syndrome characterized by ineffective intestinal propulsion with signs and symptoms similar to mechanical bowel obstruction including abdominal distension, pain, nausea, vomiting, constipation and hypoactive or absent bowel sounds, but the absence of an identifiable organic obstructive lesion [3]. IpsO reflects a dysfunction of the visceral smooth muscle or the enteric nervous system or the visceral autonomic nervous system or other unclear pathological changes. IpsO may be primary or secondary to an underlying systemic disease. Secondary causes include neurologic, endocrine, and connective tissue disease (CTD) [3], [9], [25]. Among CTD, systemic sclerosis is the most common underlying disease. Secondary IpsO has been recognized as an uncommon manifestation of SLE in recent decades [4], [5], [6], [7], [8], [9], [10], [11], [12], [13], [14], [15], [16], [17], [18], [19], [20], [21], [22], [23], [24], [25], [26], [27], [28], [29]. It can manifest as a complication of the disease or, less commonly, as the initial presentation [25], [28], [30]. It usually occurs during active lupus but may manifest itself in inactive lupus [24]. It seems that most patients had a satisfactory response to corticosteroids or immunosuppressants and the diseases can be potentially reversible with conservative treatment in an early stage, but in some cases this complication evolved regardless of the underlying disease activity [3], [23], [24], [25], [28], [30]. Physician awareness and early diagnosis could positively affect outcomes of this syndrome, avoiding unnecessary surgical intervention.

There have been 42 cases of IpsO associated with SLE to date in the English literature. The clinical profile, immunological features, histological findings, treatment modality and disease outcome of these patients are reviewed. Possible underlying mechanisms of this complication are discussed.

Section snippets

Methodology

An English literature search revealed 28 articles with 42 patients satisfying the American College of Rheumatology (ACR) revised criteria for the classification of SLE [31] who had IPO as a clinical manifestation of their underlying lupus. Patients fulfilling less than 4 of the ACR criteria were not included in our revision, although the diagnosis of SLE was highly suggestive. The disease activity in SLE was assessed according to the most widely used criteria of ECLAM or SLEDAI [32]. The review

General clinical characteristics

The characteristics of all 42 cases are summarized in Table 1. The female to male ratio was 38:4. Twenty-two (52.4%) patients had IpsO as the initial presentation of their underlying lupus. Three (7.1%) patients manifested as inactive lupus. The duration of abdominal symptoms before admitted ranged from 3 days to 3 years, however most of the patients responded well to systemic corticosteroid or immunosuppressive treatment within 2 days to about 3 months. The small bowel was more frequently involved

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 [33]. 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. Gastrointestinal complications have been increasingly observed as manifestations of SLE, and occur in 50% of them

Conflicts of interest

The authors declare that they have no conflict of interest.

Acknowledgments

This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

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