Protein-Losing Enteropathy in Systemic Lupus Erythematosus: 12 Years Experience from a Chinese Academic Center

Abstract

ObjectiveProtein-losing enteropathy (PLE) is a complication in some systemic lupus erythematosus (SLE) patients that is often misdiagnosed. With this study, we provide insight into clinical characteristics, laboratory characteristics, diagnostic tests, risk factors, treatment, and prognosis of the disease.MethodsA retrospective, case-control study was performed in 44 patients with SLE-related PLE (PLE group) and 88 patients with active SLE (control group) admitted to our care from January 2000−January 2012. Risk factors for SLE-related PLE were examined, and we analyzed the accuracy of single and combined laboratory characteristics in discriminating SLE-related PLE from active SLE. Serum albumin and C3 levels were measured as outcome during and after treatment with corticosteroids and immunosuppressive agents.ResultsThe PLE group had lower mean serum albumin and 24-hour urine protein levels, higher mean total plasma cholesterol levels, and greater frequencies of anti-SSA and SSB seropositivity compared with the control group. Anti-SSA seropositivity, hypoalbuminemia, and hypercholesterolemia were independent risk factors for SLE-related PLE. The simultaneous presence of serum albumin (<22 g/l) and 24-hour urine protein (<0.8 g/24 h) had high specificity, positive predictive value, negative predictive value, and positive likelihood ratio, a low negative likelihood ratio and no significant reduction in sensitivity. High dosage of glucocorticosteroid combined with cyclophosphomide were mostly prescribed for SLE-related PLE.ConclusionSLE-related PLE should be considered when an SLE patient presents with generalized edema, anti-SSA antibody seropositivity, hypercholesterolemia, severe hypoalbuminemia, and low 24-hour urine protein levels. Aggressive treatment for lupus might improve prognosis.

Protein-losing Gastroenteropathy Related to Mixed Connective Tissue Disease: A Case Report of a Successful Outcome and Literature Review

“…The present case showed leukocytopenia and lymphadenopathy as SLE-like findings along with sclerodactyly and pulmonary fibrotic changes as systemic sclerosis (SSc)-like findings, but general edema and abdominal distension ascribable to albumin leakage were eventually the principal symptoms determining the diagnosis of MCTD in this patient. SLE is a relatively common cause of PLGE associated with autoimmune disease, and the prevalence of PLGE with SLE has been reported to range from 0.94% to 7.5% ( 2 , 5 , 6 ). SSc is also recognized as a cause of PLGE ( 7 , 8 ).…”

“…To establish the diagnosis of PLGE and monitor the efficacy of treatment, α1-AT and 99m Tc-HAS are commonly used. The latter is especially useful for detecting the location of albumin leakage ( 2 , 6 , 15 ). On reviewing PLGE related to SLE, the small intestine seems to be the most common site of albumin leakage, while the stomach is a rare site, with a leakage frequency of up to 9%, as determined using 99m Tc-HAS ( 2 , 5 ).…”

“…With regard to the histopathological analysis of PLGE related to MCTD, four patients including the present one showed lymphocytic and/or plasmocytic infiltration, three had mucosal edema, two had lymphangiectasis, and one had immune complex deposition ( Table ). PLGE related to autoimmune diseases, including SLE and SSc, has been proposed to occur because of mucosal capillary permeability derived by complement-mediated or cytokine-mediated vascular damage, non-necrotizing vasculitis, and mucosal lacteal due to lymphangiectasis ( 3 , 6 - 8 , 16 ); however, the pathomechanism of PLGE related to MCTD remains unclear. A previous study found that the most frequent finding in PLGE associated with SLE was inflammatory cell infiltration (73%); normal cellular morphology had a frequent of 20%, and lymphangiectasis and vasculitis had frequencies of 15% and 2%, respectively ( 2 ).…”

CHAPLE syndrome uncovers the primary role of complement in a familial form of Waldmann's disease

“…Apart from the reports on aHUS and PIL discussed above, there have been descriptions of PLE in systemic disorders associated with hypocomplementemia, in particular, Lupus. PLE has long been known as a rare complication of systemic lupus erythematosus (SLE), which is recently termed as Lupus protein‐losing enteropathy (LUPLE), with a point prevalence of 3.2% to 7.5% in large series of Asian Lupus patients . The exact pathogenesis of the PLE in SLE is still unclear but blamed mechanisms include; (a) mesenteric or intestinal vasculitis, (b) complement‐mediated vessel injury, (c) IL, and (d) increased endothelial permeability .…”

A Case of Protein Losing Enteropathy as Only Clinical manifestation of Systemic Lupus Erythematosus

“…13 Chen Z et al reported that most of patients with PLE associated with SLE responded well to the combined therapy of prednisolone and cyclophosphamide. 16 However, it remains unclear whether steroid monotherapy or combination therapy with immunosuppressants is more appropriate for PLE associated with SLE patients. In this case, patient was initially treated with high dose steroid and then tapered with azathio- prine and achieved a good clinical response.…”