Background/Purpose: Protein-losing enteropathy (PLE) is a rare condition characterized by a loss of serum protein into the gastrointestinal tract resulting in hypoproteinemia, which can be complicated by edema, ascites, pleural and pericardial effusions, and malnutrition. Although rare, PLE may complicate a variety of diseases, most commonly cardiac or gastrointestinal conditions, and less infrequently, systemic autoimmune diseases (SAD).

Methods: In January 2016, we created a retrospective multicenter collection of cases of PLE reported in patients with SAD through the Spanish Autoimmune Diseases Study Group (GEAS-SEMI), and we carried out a systematic literature review. PLE was clinically diagnosed in patients presenting with hypoalbuminemia due to gastrointestinal loss after discarding urinary protein loss and reduced protein synthesis (malnutrition, liver disease, etc). Patients in whom evidence of protein leakage from the gastrointestinal tract as detected by 99 m Tc-HSA scintigraphy were classified as definite PLE, and those patients in which this examination was not performed were classified as probable PLE.

Results: A total of 263 patients with SAD-related PLE (6 patients from the Registry and 210 from the literature review) were included (80% were women, with a mean age at diagnosis of disease of 41.1 years, range 16-79 years); 226 (86%) of cases were reported from Asian countries (64% from China), 24 from European countries and 13 from America (7 from US). The main underlying SAD was systemic lupus erythematosus (SLE) in 202 (77%) cases, followed by Sjögren syndrome in 18 (7%) and sarcoidosis in 15 (6%). The main signs and symptoms at presentation were peripheral edema in 192 (85%) patients, pleural effusion in 129 (57%), ascites in 120 (53%) and diarrhea in 96 (43%) patients. Internal organ involvement CNS (in 8 (16%), pulmonary in 23 (46%) and renal in 15 (30%). The main analytical markers consisted of hypercholesterolemia (90%), hypertriglyceridemia (85%) and positive alpha1-antitrypsin in stool (68%). The main gastrointestinal endoscopic finding consisted of edematous mucosa either in upper endoscopy (67/129, 52%) and lower endoscopy (74/139, 53%). One hundred and eighty-two (69%) patients were classified as definite PLE and 81 (31%) were classified as probable PLE. Therapies included corticosteroids in 162 patients and immunosuppressive agents in 171 (mainly cyclophosphamide and azathioprine); biological agents were used in 5 patients (rituximab in 4, infliximab in 1). After a mean follow-up of 15 months, 77% of patients had a complete response, 6% developed a disease relapse, and 4% died.

Conclusion: A very specific epidemiological and clinical profile was observed for protein-losing enteropathy associated with systemic autoimmune diseases, with nearly 90% of cases reported from Asian countries (two out of three cases from China), and nearly 80% reported in patients with underlying SLE. Patients were overwhelmingly treated with corticosteroids and immunosuppressive agents with a complete response of nearly 80% of cases and a global mortality of less than 5%.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/protein-losing-enteropathy-in-patients-with-systemic-autoimmune-diseases-characterization-of-263-cases-geas-semi-spanish-cohort/