Sjögren protein-losing enteropathy: a systematic review
DOI:
https://doi.org/10.36303/SAJGH.2604Keywords:
Sjögren syndrome, protein-losing enteropathyAbstract
Background: Sjögren protein-losing enteropathy (SPLE) is a rare manifestation of Sjögren syndrome (SS). This study aimed to identify the characteristics of patients with SPLE.
Methods: A systematic review was performed with 22 patients who met the eligibility criteria and were critically appraised. Technetium-99m (99mTc) albumin scintigraphy or a faecal alpha-1 antitrypsin (A1AT) clearance test was ultimately used to identify SPLE.
Results: Patients’ clinical features at the time of SPLE diagnosis were 51.8 ± 16.3 years, and the female-to-male ratio was 8.1:1. In most cases (86%), the SS diagnosis was retained as part of the aetiological investigation of protein-losing enteropathy (PLE). Serologically, anti-Ro was present in all cases and anti-La in 49%.
Conclusion: In this review, 15 cases underwent 99mTC human serum albumin (HSA). Protein leakage was positive in all the above cases. A 24-hour stool specimen showed a positive faecal A1AT clearance test. This is the first systematic review of SPLE associated with SS.
