Acute liver failure as the first feature of systemic lupus erythematosus
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Rheumatology INTERNATIONAL
CASE BASED REVIEW
Acute liver failure as the first feature of systemic lupus erythematosus Mohammad Mustafa1 · Yasser Mohammed Bawazir2 Received: 14 August 2020 / Accepted: 28 September 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease. Although gastrointestinal (GI) involvement is reported in the literature, GI manifestations of lupus are not common in the early disease course. GI symptoms can be related to several factors other than lupus; however, systemic lupus per se can be the responsible factor. Although the typical presentation is a gradual onset of progressive symptoms, acute abdominal pain is frequently reported and is the most serious presentation. It can reflect gastroenteritis, cholecystitis, hepatitis, pancreatitis, peritonitis, and abdominal vasculitis. When SLE diagnostic criteria for GI manifestations are lacking, the decision to implicate lupus as the cause of these manifestations is difficult, especially in the primary presentation. Early diagnosis and the initiation of immunosuppressive agents are associated with a better outcome. In this case, we introduce a patient who presented with acute abdominal pain secondary to acute liver failure as the first manifestation of lupus. Keywords Systemic lupus erythematous · Acute liver failure · Lupus pancreatitis · Lupus hepatitis · Lupus enteritis
Introduction Systemic lupus erythematosus (SLE) is an autoimmune disease that typically affects young women and is characterized by widely different patterns of presentation and involvement of multiple systems, including the musculoskeletal, mucocutaneous, pulmonary, cardiac, renal, hematological, and neurovascular systems [1]. The presence of immunological markers of systemic lupus, such as antinuclear antibodies (ANA), anti-double-strand antibodies (anti-ds DNA), and anti-Smith antibodies (Anti-sm), and low complement, in conjunction with clinical features, is usually required for SLE diagnosis [2]. Disease severity varies from mild cases to life-threatening conditions. The SLE disease activity index (SLEDAI) score is a validated standard score that is used to assess SLE disease activity. It is composed of 24 lupus features; each one, if present, is scored on a scale from * Mohammad Mustafa [email protected] Yasser Mohammed Bawazir [email protected] 1
Department of Medicine, University of Jeddah, Jeddah, Saudi Arabia
Department of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia
2
1–8, with a maximum total score of 105 [3]. SLE is considered inactive if SLEDAI ≤ 2, while SLEDAI = 3 or greater indicates active disease [4]. Gastrointestinal (GI) manifestations are common in SLE, with a prevalence ranging from 25 to 60% [5]. The most commonly presented symptoms are nausea and vomiting (53%) and anorexia (49%), followed by abdominal pain (19%). Medication side effects, infections, and disease complications are the major contributing factors for GI symptoms [6,
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