Selective plasma exchange for the treatment of Stevens-Johnson syndrome, toxic epidermal necrolysis, and anti-glomerular basement membrane disease

Abstract

Blood purification through plasmapheresis therapy is often performed in critical care to treat Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and anti-glomerular basement membrane disease (anti-GBM disease). A simple plasma exchange using fresh frozen plasma as the replacement fluid is used particularly for diseases with bleeding, such as alveolar hemorrhage, as it supplements coagulation factors. However, the use of large amounts of fresh frozen plasma may cause complications, including allergies. Selective plasma exchange is a type of plasma exchange that uses a selective membrane separator to effectively remove pathogenic substances of smaller molecular weight than immunoglobulin G, while retaining the coagulation factors, such as factor XⅢ and fibrinogen, even when an albumin solution is used as the replacement fluid. Although the effectiveness of selective plasma exchange has been reported in immune-mediated disorders, there is little information regarding its use to treat SJS/TEN and anti-GBM disease. The present study presents an overview on the use of selective plasma exchange for the treatment of SJS/TEN and anti-GBM disease.