Protein-Losing Gastroenteropathy Associated With Sjögren's Syndrome: First Known Case Reported Outside of Asia

Abstract

Protein-losing gastroenteropathy (PLGE) is a rare extraglandular manifestation of Sjögren's syndrome, reported in fewer than 10 cases. We report a 58-year-old white woman with Sjögren's syndrome, type 1 renal tubular acidosis, and PLGE, who presented with cachexia and 100-pound weight loss. The diagnosis was made based on hypoalbuminemia in the absence of significant proteinuria, low levels of fat soluble vitamins, low transferrin, and an elevated alpha-1 antitrypsin (A1AT) fecal clearance, supported by imaging and endoscopy, with biopsy showing lymphocytic infiltration. She was successfully treated with cyclophosphamide and prednisone. To our knowledge, this is the first such case outside of Asia.

Sjögren's syndrome concurrent with protein‐losing gastroenteropathy with secondary systemic capillary leak syndrome : A case report

“…Autoimmume diseases that cause protein‐losing gastroenteropathy include scleroderma, systemic lupus erythematosus, Sjögren's syndrome, rheumatoid arthritis, mixed connective tissue disease, and dermatomyositis, and the cause of protein‐losing gastroenteropathy associated with autoimmune disease is thought to be related to capillary hyperpermeability . Among autoimmune diseases, there have been 21 published reports from 1988 to 2017 in English and Japanese of cases presenting with Sjögren's syndrome concurrent with protein‐losing gastroenteropathy, as in the present case (Table ) . Among such publications, there are no reports of secondary systemic capillary leak syndrome, thereby making our case the first case to be reported.…”

“…1 Among autoimmune diseases, there have been 21 published reports from 1988 to 2017 in English and Japanese of cases presenting with Sjögren's syndrome concurrent with proteinlosing gastroenteropathy, as in the present case (Table 2). 1,[4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] Among such publications, there are no reports of secondary systemic capillary leak syndrome, thereby making our case the first case to be reported. Among the 21 reported cases, 18 were females and 19 were reported from East Asia.…”

A case report of Sjögren’s syndrome associated with protein-losing gastroenteropathy successfully treated with methylprednisolone

“…21 There is no standard treatment for PLGE associated with Sj€ ogren's syndrome, and treatment options include glucocorticoids, hydroxychloroquine, cyclophosphamide and rituximab. [6][7][8][9][10][11][12]15 In general, as in the present case, patients tend to respond favourably to glucocorticoids. However, some steroid-resistant patients require other immunosuppressive agents, for example, cyclophosphamide.…”

“…However, some steroid-resistant patients require other immunosuppressive agents, for example, cyclophosphamide. 10 , 15 It has also been reported that rituximab has completely alleviated symptoms in some patients who are resistant to steroid and cyclophosphamide treatments. 8 …”

“…This report described the case of an elderly Chinese male patient with primary Sjögren’s syndrome in association with PLGE in mainland China. A review of the literature revealed that most cases of PLE associated with primary Sjögren’s syndrome have been reported in patients from East Asia, particularly Japan ( Table 1 ), 6 – 12 , 15 and the disease is uncommon in mainland China. PLGE was suspected in the present case following exclusion of other factors causing low protein, such as liver dysfunction, nephrotic syndrome, cardiovascular disease, malnutrition and cachexia.…”