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

Abstract

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.

Immune-mediated Protein-losing Enteropathy with Down Syndrome

“…Lymphangiectasia was not revealed by histological studies in the present case. Although marked vasculitis was not observed, vasculitis was surely induced by products of serum complement C3 conversion and caused increased intestinal vessel permeability (14), as we observed extreme consumption of serum complements and IgM and complement C3 deposition in the capillary walls of the lamina propria. We cannot eliminate the possibility of capillary leakiness for proteins induced by various cytokines, such as interleukin-2 produced by T lymphocytes infiltrating the lamina propria (15).…”

“…Similarly, hypocomplementemia occurs in PLE associated with SLE (14). However, her clinical presentation and laboratory findings suggested that she had immunemediated PLE.…”

Chronic Diarrhea Unmasking Systemic Lupus Erythematosus

“…Colonoscopy is often normal and thus nondiagnostic, although can demonstrate mucosal thickening. Up to 80% of patients have histological findings of mucosal edema, inflammation, lymphangiectasia, mucosal atrophy, or vasculitis [7].…”

Gastrointestinal manifestations associated with systemic lupus erythematosus

“…Jest to zespół objawów klinicznych wynikających z nadmiernej utraty białek osocza do światła jelita, związanej ze zmianami morfologiczno--czynnościowymi naczyń chłonnych lub błony śluzowej ścia-ny jelita. Na obraz kliniczny składają się: przewlekła, czę-sto tłuszczowa biegunka, nudności i wymioty, obrzęki w przebiegu hipoalbuminemii, wodobrzusze i niedożywienie oraz objawy niedoboru witamin A i D [16,29]. Tsutsumi i Sunheimer opisali ponad 20 przypadków pacjentów z enteropatią z utratą białka w przebiegu TRU [30,31].…”