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The clinical characteristics of lupus related protein-losing enteropathy in Hong Kong Chinese population: 10 years of experience from a regional hospital
Lupus, 06/01/2012
Clinical Article
Law ST et al. – There appears to be increasing prevalence of systemic lupus erythematosus (SLE) related protein–losing enteropathy (PLE). A diagnosis can be made using 99m Tc–labeled HSA scintigraphy. PLE generally responds well to immunosuppressive therapy.
Methods- A retrospective analysis was performed.
- From 2001 to 2010, 48 patients had SLE related PLE and their clinical characteristics were: age 40.8 ± 14.3 years, male–to–female ratio 1:8.6, mean symptom duration 4.3 ± 3.4 weeks, initial presentation and concomitant activity of SLE in 21(43.8%) and 37 (77.1%) patients, <20% patients developed gastrointestinal (GI) symptoms, mean serum albumin level 24.4 ± 5 g/L.
- Thirty (62.5%) patients had diffuse non–erosive erythematous GI mucosa with chronic inflammatory cells in lamina propria.
- Protein leakage was at the small bowel in 15 (31.3%) patients, terminal ileum/caecum in 16 (33.3%) patients and ascending colon in 11 (22.9%) patients.
- Thirty (62.5%) patients responded initially well to a combination of prednisolone and azathioprine (AZA) and 33 (68.8%) patients were maintained well by the above therapy.
- Higher potent induction and maintenance therapy were required in patients with: proteinuria (p < 0.01), history of previous immunosuppressive therapy (p < 0.02) and requirement of higher potent induction therapy (p < 0.01).
- PLE as initial SLE presentation was associated with better prognosis.
- Four reversible adverse events were reported: one had AZA–induced pancreatitis, two developed AZA–induced hypoplastic anemia and one developed steroid psychosis.
- One patient developed shingles in the fourth month and responded to oral acyclovir.
- No thromboembolic events were reported and one patient died of SLE nephropathy.