Isolated Lupus-Associated Protein-Losing Enteropathy in a Resource-Limited Centre

Systemic lupus erythematosus (SLE) is a relatively common autoimmune disease with recently reported cases of lupus-associated protein-losing enteropathy (LUPLE) as an unusual manifestation. It is a well-recognized clinical entity predominantly affecting middle-aged Asian females. LUPLE is diagnosed by exclusion of possible causes for hypoalbuminemia in a patient with positive anti-nuclear antibody (ANA). LUPLE as the first manifestation of SLE is rare but it is a well-recognized complication secondary to SLE. We report a case of a 39-year-old Sri Lankan lady who was investigated for generalized body swelling, pleural effusions, ascites and pericardial effusions due to hypoalbuminemia. Her ANA was positive with speckled pattern and intestinal biopsy samples revealed evidences of chronic inflammatory cell infiltrates in laminapropria. Her investigations were not suggestive of liver diseases, albuminuria or malnutrition. We excluded all possible etiologies for protein-losing enteropathy although gold standard tests to confirm it was not available in our center. In conclusion, LUPLE should be considered as an etiology for all the unexplained protein-losing enteropathies. We suggest to treat LUPLE with prednisolone, hydroxychloroquine (HCQ) followed by steroid-sparing agents such as azathioprine. Prognosis was excellent following appropriate treatment.