Successful identical-twin living donor small bowel transplant for necrotizing enterovasculitis secondary to Churg-Strauss syndrome

Churg-Strauss syndrome (CSS) is a granulomatous small-vessel vasculitis with unknown etiology. Extra-pulmonary manifestations of CSS are currently treated with a combination of steroids and Cyclophosphamide. Its gastrointestinal complications may be devastating, occasionally requiring extensive bowel resection resulting in short-gut syndrome. Living-related small bowel transplantation (LRSBTx) is a relatively standardized procedure that, not only represents a valid alternative to cadaver bowel transplant in selected cases, but also portraits excellent results when performed in experienced centers. The availability of an identical twin as a donor, which allows avoidance of immunosuppressive therapy, is a major indication for this procedure. We present the case of a young individual affected by gastrointestinal necrotizing vasculitis that lost almost his entire small bowel requiring the immediate institution of total parenteral nutrition (TPN). However, within few weeks a significant hepatic dysfunction ensued. An identical twin-brother, not affected with CSS, became an immediate, optimal donor-candidate for LRSBTx, the first of this kind in a patient affected with CSS. Following the procedure, two main concerns were addressed: the recipient's ability to recover a regular intestinal function without immunosuppression and the possible recurrence of the primary disease. Twenty-seven months post-transplant, the patient enjoys a regular lifestyle without any clinical, endoscopic and histologic evidences of recurrent disease in the transplanted graft.