The Role Of Factor Xiii And Elastase In Gastrointestinal Complications In Henoch-Schoenlein Purpura

Henoch-Schoenlein purpura (HSP) is a generalized anaphylactoid ''leucocytoclastic'' vasculitis, which in more than 50% of patients is complicated by gastrointestinal symptoms such as vomiting, abdominal pain, melena and life-threatening bleedings. In five consecutive patients with HSP on admission factor XIII (F XIII) was decreased to 8-58% of normal. In acute attacks an enormous F XIII consumption takes place. Recurrent release of elastase from inflammatory cells (PMNL, monocytes, macrophages) can be detected by intermittent peaks of elastase-alpha(1)-protease inhibitor (ELP-alpha(1)-PI) complex in plasma (peak values 450-950 ng/ml). Elastase is considered to cause specific F XIII degradation with bleeding into the intestinal wall as a result. High-dose F XIII substitution therapy is effective in controlling bleeding complications and abdominal pain. The benefit of F XIII therapy could be demonstrated in a rapid and marked shrinking of the intramural hematomas by ultrasonic scan.