The Digestive Involvement in Systemic Autoimmune Diseases

Manuel Ramos-Casals, Munther Khamashta, Pilar Brito-Zeron, Fabiola Atzeni, Joan Rodes

• Publisher: Elsevier
• Year: 2017
• eBook ISBN: 9780444637178
• Edition: 2
• Topic: Medicina
• Subtopic: Nutrizione, dietetica e bariatria

The Digestive System in Systemic Autoimmune Diseases, Second Edition, represents the state-of-the-art in the field of digestive disorders in the most common systemic autoimmune diseases. This volume consists of an introductory chapter on imaging techniques in digestive diseases, followed by eight chapters on digestive manifestations in specific systemic autoimmune diseases. The final five chapters deal with digestive diseases with an autoimmune pathogenesis and systemic manifestations. International in scope, the table of contents reads like a Who's who in clinical research on systemic autoimmune diseases. More than 20 contributors from the European Union, the United States, Mexico, and South Africa share their knowledge in this detailed volume. The book provides an overview of our current understanding of digestive disorders in the most common systemic autoimmune diseases.- Completely updated, including five new chapters- Presents up-to-date information, giving the reader easy access to individual topics in one place- Written by leading international clinical and scientific experts on autoimmune and digestive diseases- Provides a practical guide to the identification, diagnosis, and treatment of digestive involvement in patients with autoimmune diseases that will be useful for all medical specialties- Includes several diseases and conditions not included in other texts, some of which have only recently emerged- Designed to serve as a guide to clinical practice

Section IV Gastrointestinal Involvement of Systemic Diseases

Outline

Chapter 11. Systemic Lupus Erythematosus

Chapter 12. Digestive Involvement in the Antiphospholipid Syndrome

Chapter 13. Gastrointestinal Involvement in Systemic Sclerosis

Chapter 14. Gastrointestinal Involvement in Inflammatory Myositis

Chapter 15. Digestive Involvement in Primary Sjögren's Syndrome

Chapter 16. Gastrointestinal Involvement in Systemic Vasculitis

Chapter 17. Mixed Connective Tissue Disease

Chapter 18. Gastrointestinal Manifestations of Rheumatoid Arthritis

Chapter 19. Spondyloarthritis and Gastrointestinal Involvement

Chapter 20. Intestinal Behçet's Disease

Chapter 21. Gastrointestinal Involvement of Sarcoidosis

Chapter 22. IgG4-Related Disease: Gastrointestinal Involvement

Chapter 23. Gastrointestinal Complications of Antirheumatic Drugs

Chapter 11

Systemic Lupus Erythematosus

M. Vilardell-Tarrés, A. Selva-O'Callaghan and J. Ordi-Ros Vall D'Hebron General Hospital, Barcelona, Spain

Abstract

Gastrointestinal involvement affecting the oral cavity, esophagus, stomach, small bowel, or colon is common in systemic lupus erythematosus (SLE) and occasionally life-threatening if not treated promptly. Abdominal pain is sometimes the only symptom, making the diagnosis a challenge for clinicians. Usually, the clinical gastrointestinal symptoms run in parallel with SLE disease activity. Abdominal CT is useful for establishing a prompt diagnosis, as it enables visualization of the vasculature and can depict bowel abnormalities, mesenteric edema, and ascites. Chronic intestinal pseudoobstruction, protein-losing gastroenteropathy, and intestinal vasculitis are the most common identifiable SLE-related gastrointestinal manifestations. The precise pathogenesis of these conditions is uncertain, but several disease- and treatment-related factors may be implicated, such as vasculitis, cytokine-mediated damage, complement deposition, and the presence of antiphospholipid antibodies. Immunosuppression with prednisone, adding cyclophosphamide in refractory cases, is the cornerstone therapy for most SLE-related gastrointestinal manifestations. If the patient's general condition deteriorates, surgery is occasionally recommended.

Keywords

Chronic intestinal pseudoobstruction; Lupus mesenteric vasculitis; Pneumatosis cystoides intestinalis; Protein-losing enteropathy; Systemic lupus erythematosus

1. Introduction

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with a broad range of clinical manifestations. It is characterized by an immune system dysregulation resulting in the production of various autoantibodies and is considered a multifactorial disease with evidence of genetic susceptibility [1]. SLE affects several organ systems and leads to significant morbidity and mortality. Gastrointestinal (GI) manifestations caused by the disease per se or aggressive treatment regimens are not rare in SLE patients [2], but are seldom reported, likely being masked by other, more salient clinical features such as renal or central nervous system abnormalities. The incidence of GI symptoms attributable to the disease itself varies widely, ranging from 1.3% to 27.5% in the literature. Chronic intestinal pseudoobstruction (CIPO), protein-losing gastroenteropathy, and intestinal vasculitis are the most common identifiable SLE-related GI manifestations [3], which seem to occur more commonly in Asian populations. No specific autoantibodies associated with SLE-related gastroenteropathy have been identified to date. In this chapter, we describe the GI manifestations occurring in SLE patients. The liver manifestations of the disease are beyond the scope of this chapter and will be reviewed elsewhere.

2. Overview of Gastrointestinal Manifestations in Systemic Lupus Erythematosus

Almost half of all lupus patients experience anorexia, nausea, and vomiting. These symptoms may result not only by GI involvement but also from the uremia associated with renal failure, or from the effects of cytostatic therapies, such as azathioprine and intravenous cyclophosphamide pulses, or the more recently used immunosuppressive drug, mycophenolate mofetil.

Serositis (pleuritis, pericarditis, and less frequently, ascites) is a well-recognized diagnostic criterion of SLE. It is present in 8–11% of patients and can be divided into inflammatory (true serositis) or noninflammatory serositis, mainly caused by the hypoalbuminemia resulting from nephrotic syndrome, liver cirrhosis, or protein-losing enteropathy. Chronic ascites due to lupus per se has a painless onset, progresses even in the absence of other signs of disease activity, and usually responds to corticosteroids or in refractory cases, cyclophosphamide [4,5].

GI symptoms are the first complaints in nearly one-third of lupus patients, although they can occur at any stage of the disease. It is important to be aware of these manifestations in SLE because misdiagnoses and delayed treatment can lead to unfavorable outcomes [3].

Abdominal pain is usually a nonspecific symptom, but it can be particularly important in lupus patients, occurring in adults with this condition [4] and in childhood-onset SLE [6]. Acute abdomen is always a challenging diagnostic and therapeutic problem—even more so in SLE [7]—and the abdominal pain may also be caused by the complications of therapy or the disease, itself (Table 11.1). Immunosuppressive agents and corticosteroids, the usual treatments for SLE, may mask the classic symptoms of bowel perforation and ischemia, two of the main causes of acute abdomen. Nonsteroidal antiinflammatory drugs, azathioprine, calcineurin antagonists such as cyclosporine and tacrolimus, and mycophenolate mofetil can all cause abdominal pain to a greater or lesser extent. Evaluation of the patient's disease activity using the SLEDAI (Systemic Lupus Erythematosus Disease Activity Index) may help physicians to choose the most effective approach. Intraabdominal vasculitis and mesenteric thrombosis, both severe abdominal complications of SLE, seem to be associated with higher scores on the SLEDAI [5] and other activity indices [8]. However, one study has reported that the SLEDAI is not reliable as a general marker of SLE-related GI manifestations [9]. Radiological examinations such as ultrasound and computed tomography (CT) scanning are essential to achieve the correct diagnosis; gastroscopy and colonoscopy, which can show ischemia and ulcers, are also of value. Prompt laparotomy is preferred in some cases, particularly acute abdomen with active SLE or a severe clinical presentation, because mortality is high in these patients [10,11].

Table 11.1

Abdominal Pain in Systemic Lupus Erythematosus (SLE)

SLE-Related	Treatment-Related	Non-SLE Causes
Renal vein thrombosis	Gastritis, duodenitis	Appendicitis
Mesenteric thrombosis	Pancreatitis (azathioprine)	Viral hepatitis
Acalculous cholecystitis	Sepsis	Biliary pancreatitis
Bowel perforation	Peptic ulcer	Diverticulitis
Vasculitis	Perforation	Surgical adhesions
Ectopic pregnancy	Enterocolitis
Pancre...

Table of contents

• Cover image
• Title page
• Table of Contents
• Handbook of Systemic Autoimmune Diseases
• Copyright
• Dedication
• List of Contributors
• Preface
• Section I Introduction
• Section II Autoimmune Liver Diseases
• Section III Autoimmune Manifestations of Viral Hepatitis
• Section IV Gastrointestinal Involvement of Systemic Diseases
• Index