Background/Purpose: Protein losing enteropathy (PLE), characterized by severe hypoalbuminemia and edema, is rare manifestation of systemic lupus erythematosus (SLE). The study was proposed to identify the distinct features of lupus PLE and to evaluate the factors related with treatment response or outcome of lupus PLE.

Methods: From Mar. 1998 to Mar. 2014, the clinical data of 14 patients with lupus related PLE (lupus PLE) and 7 patients with idiopathic PLE in tertiary center were reviewed. PLE was defined as demonstration of protein leakage from gastrointestinal tract by either technetium 99m-labelled human albumin scan or fecal α1-antitrypsin clearance with no evidence of protein loss from other sources and impaired protein synthesis. Positive steroid response (PSR) was defined as return of serum albumin to ≥ 3.0 g/dl within 4 weeks after initial steroid monotherapy and remission as maintenance of serum albumin ≥ 3.0 g/dl for at least 3 months. High total cholesterol means the serum total cholesterol level of ≥ 240 mg/dl.

Results: The mean age of lupus PLE was 37.0 ± 19.8 years (range: 16 – 72) and 11 patients (78.6%) had PLE as initial manifestation of SLE. The mean follow-up duration was 55.8 ± 16.0 months (range: 6 – 172). There were significant increases in ESR and total cholesterol level in lupus PLE compared with idiopathic PLE. Among 14 patients with lupus PLE patients, 8 patients experienced PSR. Total cholesterol level was significantly higher in PSR group (Table 1). PSR was associated with initial high total cholesterol (OR = 7.0, 95% CI = 1.14 – 42.97) and with achievement of remission in 6 months (OR = 3.0, 95% CI = 0.97 – 9.30). Among 14 patients with lupus PLE, 10 patients who achieved remission in 6 months showed higher total cholesterol level (283.3 ± 79.3 mg/dL) compared to 4 patients who did not (165.3 ± 63.9 mg/dL).

Conclusion: In lupus PLE, high total cholesterol level could be a predictive factor to initial steroid response, expecting good response to steroid therapy alone. Furthermore, we suggest that initial high level of total cholesterol could predict a favorable outcome in patient with lupus PLE.

Table 1 Characteristics of lupus related PLE patients according to steroid response

Characteristic	Positive steroid response (n=8)	Negative steroid response (n=6)
Age, years	37.0 ± 19.8	48.3 ± 7.7
Sex, male (%)	4 (50)	2 (33.3)
Symptom duration before treatment, weeks	6.1 ± 4.5	16.0 ± 11.5
White blood cells, x10³/ul	7.7 ± 3.3	7.0 ± 1.9
Lymphocyte, x10³/ul	1.9 ± 0.7	1.5 ± 0.5
Hemoglobin, g/dl	12.8 ± 1.1	11.5 ± 1.5
Platelets, x10³/ul	202.8 ± 119.9	260.1 ± 107.2
ESR, mm/h a	68.3 ± 23.9	70.7 ± 51.2
CRP, mg/dl	0.9 ± 1.9	1.1 ± 1.0
C3, mg/dl	52.8 ± 24.6	44.3 ± 19.4
C4, mg/dl	11.3 ± 5.7	13.2 ± 3.2
Anti-ds DNA antibody, IU/ml	16.6 ± 19.8	20.4 ± 41.5
SLEDAI score	6.5 ± 3.3	7.67 ± 3.7
Protein, g/dl	4.2 ± 1.6	4.23 ± 0.9
Albumin, g/dl	1.3 ± 0.5	1.2 ± 0.35
Total cholesterol level, mg/dl a	304.6 ± 74.0	176.6 ± 52.3
High total cholesterol, (%)	7 (87.5)	0 (0)
Remission within 6 months (%)	8 (100)	2 (33.3)

^a: p < 0.05

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/protein-losing-enteropathy-in-systemic-lupus-erythematosus/