Lupus protein-losing enteropathy (LUPLE): A systematic review.

Summary of "Lupus protein-losing enteropathy (LUPLE): A systematic review."

Lupus protein-losing enteropathy (LUPLE) is a well reported but a rare manifestation of systemic lupus erythematosus (SLE). The main objectives of this study are to raise awareness of LUPLE that can be easily missed by internist, rheumatologist, gastroenterologist and nephrologist, and then to be considered in any patient with unexplained edema, ascites, and hypoalbuminemia. A systematic review was performed with 112 patients who met the eligibility criteria and were critically appraised. The LUPLE was ultimately diagnosed by either Tc-(99m) albumin scintography ((99m)Tc-HAS) or fecal alpha-1-antitrypsin clearance test. Clinical features of patients, at the time of LUPLE diagnosis, were as follows: age was 34 ± 14.2 years; the female to male ratio was 5.8:1; the mean time to development of LUPLE after diagnosis of SLE was 4.19 ± 4.7 years. There was a predominance of Asian (64.7%) while 29.5% were white or Hispanic patients. Eighty percent had peripheral edema, 48% had ascites, 38% had pleural effusion, and 21% had pericardial effusion. Forty-six percent had diarrhea, 27% had abdominal pain, 22% had nausea, and 19% had vomiting. Hypoalbuminemia was the most common characteristic laboratory finding (96%). A 24-h urine protein was less than 0.5 gm in (71%). Almost all patients (96%) had positive ANA with predominant speckled patterns (55%) and hypocomplementemia (79%). Colonoscopy showed mucosal thickening in 44% of patients, and the majority of patients (52%) revealed no abnormalities; on the other hand, intestinal histology either revealed mucosal edema, inflammatory cell infiltrate, lymphangiectasia, mucosal atrophy or vasculitis in 80% of patients. All patients were started on steroids. Thirty-four percent responded to steroids alone. Sixty-six percent were started with other immunosuppressive therapies, which include cyclophosphamide (46%), azathioprine (33%), and a combination of cyclophosphamide and azathioprine (7%). A few reported cases responded to either cyclosporine or etanercept. Prognosis was very good with steroids combined with immunosuppressive therapy. This is the first systematic review of LUPLE and should be considered as an etiology of unidentified edema, ascites, and hypoalbuminemia.


Rheumatology Division, Department of Medicine, King Saud University, Riyadh, Saudi Arabia,

Journal Details

This article was published in the following journal.

Name: Rheumatology international
ISSN: 1437-160X


DeepDyve research library

PubMed Articles [26729 Associated PubMed Articles listed on BioPortfolio]

Massive Mesenteric Lymphadenopathy Causing Protein-losing Enteropathy in Gaucher Disease: Retraction.

Medication Non-Adherence in Systemic Lupus Erythematosus: A Systematic Review.

Medication non-adherence has not been well characterized in systemic lupus erythematosus (SLE). Our objective was to a conduct a systematic review of the literature examining the burden and determinan...

Drugs for discoid lupus erythematosus.

Discoid lupus erythematosus (DLE) is a chronic form of cutaneous lupus, which can cause scarring. Many drugs have been used to treat this disease and some (such as thalidomide, cyclophosphamide and az...

Systemic lupus erythematosus and contraception: A systematic literature review.

The aim of our study was to evaluate the safety of contraceptive methods use among women with systemic lupus erythematosus (SLE).

Imaging adult patients with Fontan circulation.

Survival after the Fontan procedure for palliation of single ventricle congenital heart disease has improved. However, adults with Fontan circulation are at risk for several complications including he...

Clinical Trials [3233 Associated Clinical Trials listed on BioPortfolio]

Safety and Efficacy of ODSH in Protein Losing Enteropathy Secondary to Single Ventricle Palliative Surgery

Protein Losing Enteropathy (PLE) is a serious and sometimes fatal medical condition that may develop in subjects with congenital cardiac disease for which a palliative procedure known as t...

Using a Systematic Review in Clinical Decision Making: a Pilot Parallel, Randomized Controlled Trial

The purpose of this pilot study is to determine the feasibility of running a full-scale trial that compares two formats of a shortened systematic review to a full-length systematic review ...

Duke Lupus Registry

Lupus is a systemic autoimmune disease that can present with many varied symptoms, including joint pain, fevers, kidney disease, and rashes. Lupus can affect anyone, but it is most common...

Role of Altered CD40-Ligand Gene Transcription in Systemic Lupus Erythematosus

Systemic lupus erythematosus is an often devastating autoimmune disease which affects 1 in 2,000 women in the United States. Recently, several research laboratories have reported that a pr...

Plasma Citrulline Concentration in Tropical Enteropathy

Citrulline is an amino acid produced in the intestine and in the liver, but the liver does not contribute significantly to circulating citrulline concentrations. The intestine is thus the ...

Medical and Biotech [MESH] Definitions

Pathological conditions in the INTESTINES that are characterized by the gastrointestinal loss of serum proteins, including SERUM ALBUMIN; IMMUNOGLOBULINS; and at times LYMPHOCYTES. Severe condition can result in HYPOGAMMAGLOBULINEMIA or LYMPHOPENIA. Protein-losing enteropathies are associated with a number of diseases including INTESTINAL LYMPHANGIECTASIS; WHIPPLE'S DISEASE; and NEOPLASMS of the SMALL INTESTINE.

A form of lupus erythematosus in which the skin may be the only organ involved or in which skin involvement precedes the spread into other body systems. It has been classified into three forms - acute (= LUPUS ERYTHEMATOSUS, SYSTEMIC with skin lesions), subacute, and chronic (= LUPUS ERYTHEMATOSUS, DISCOID).

A syndrome characterized by chronic, well-established DIARRHEA (greater than one month in duration) without an identified infectious cause after thorough evaluation, in an HIV-positive individual. It is thought to be due to direct or indirect effects of HIV on the enteric mucosa. HIV enteropathy is a diagnosis of exclusion and can be made only after other forms of diarrheal illness have been ruled out. (Harrison's Principles of Internal Medicine, 13th ed, pp1607-8; Haubrich et al., Bockus Gastroenterology, 5th ed, p1155)

Review of the medical necessity of hospital or other health facility admissions, upon or within a short time following an admission, and periodic review of services provided during the course of treatment.

Multi-step systematic review process used for improving safety by investigation of incidents to find what happened, why it happened, and to determine what can be done to prevent it from happening again.

Quick Search

DeepDyve research library

Relevant Topics

Arthritis Fibromyalgia Gout Lupus Rheumatic Rheumatology is the medical specialty concerned with the diagnosis and management of disease involving joints, tendons, muscles, ligaments and associated structures (Oxford Medical Diction...

Lupus is a complex and poorly understood condition that affects many parts of the body and causes symptoms ranging from mild to life-threatening. There are several types of lupus. The main types are: discoid lupus erythematosus drug-induced lu...

Searches Linking to this Article