title: The clinical characteristics of lupus related protein-losing enteropathy in Hong Kong Chinese population: 10 years of experience from a regional hospital.
abstract: OBJECTIVE
  The aim of our study was to investigate systemic lupus erythematosus (SLE) related protein-losing enteropathy (PLE) in the following areas: clinical features, laboratory, endoscopic and imaging characteristics, treatment and outcome.
  
  
  METHODS
  A retrospective analysis was performed.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  There appears to be increasing prevalence of SLE related PLE. A diagnosis can be made using 99m Tc-labeled HSA scintigraphy. PLE generally responds well to immunosuppressive therapy.
authors.affiliation: Department of Medicine and Geriatrics, Tuen Mun Hospital, Hong Kong. stl168@hotmail.com
authors.forename: S T
authors.initials: ST
authors.lastname: Law
authors.forename: K M
authors.initials: KM
authors.lastname: Ma
authors.forename: K K
authors.initials: KK
authors.lastname: Li
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000704:Analysis of Variance; D000893:Anti-Inflammatory Agents / Q000009:adverse effects / Q000627:therapeutic use*; D001379:Azathioprine / Q000009:adverse effects / Q000627:therapeutic use*; D001799:Blood Sedimentation; D002097:C-Reactive Protein; D002432:Cecum / Q000473:pathology / Q000503:physiopathology; D016009:Chi-Square Distribution; D044682:Colon, Ascending / Q000473:pathology / Q000503:physiopathology; D016099:Endoscopy, Gastrointestinal; D005260:Female; D006723:Hong Kong; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D060828:Induction Chemotherapy; D007421:Intestine, Small / Q000473:pathology / Q000503:physiopathology; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000150:complications / Q000188:drug therapy*; D060046:Maintenance Chemotherapy; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000009:adverse effects / Q000627:therapeutic use*; D011504:Protein-Losing Enteropathies / Q000097:blood / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy*; D011877:Radionuclide Imaging; D012189:Retrospective Studies; D012709:Serum Albumin / Q000378:metabolism; D018709:Statistics, Nonparametric; D013667:Technetium; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D012709:Serum Albumin; D013667:Technetium; D002097:C-Reactive Protein; D011239:Prednisolone; D001379:Azathioprine