Protein-losing enteropathy may be an important characteristic manifestation in Sjögren’s syndrome

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.…”

“…8 In a previously published case report, PLGE was suggested to be the characteristic manifestation of primary Sj€ ogren's syndrome. 10 In conclusion, PLGE associated with primary Sj€ ogren's syndrome is a rare condition, and most reported cases have involved patients from East Asia, particularly Japan. These patients are usually characterized by profound oedema and severe hypoalbuminemia (summarised in Table 1), and always lack typical presenting symptoms of Sj€ ogren's syndrome, such as dry eye and mouth.…”

“…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 In a previously published case report, PLGE was suggested to be the characteristic manifestation of primary Sj€ ogren's syndrome.…”

“…This report described the case of an elderly Chinese male patient with primary Sj€ ogren's syndrome in association with PLGE in mainland China. A review of the literature revealed that most cases of PLE associated with primary Sj€ ogren's syndrome have been reported in patients from East Asia, particularly Japan (Table 1), [6][7][8][9][10][11][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.…”

“…1 PLGE has been associated with a wide range of illnesses, including cardiac diseases, many gastrointestinal disorders and various bacterial or viral infections, 2 and is sometimes seen to occur in autoimmune diseases, particularly systemic lupus erythematosus. [3][4][5] PLGEassociated with Sj€ ogren's syndrome is extremely rare, with most published cases coming from Japan, [6][7][8][9][10] and three cases reported from Taiwan. 11,12 To date, no cases have been reported from mainland China.…”