THE ROLE OF FACTOR-XIII AND ELASTASE IN GASTROINTESTINAL COMPLICATIONS IN HENOCH-SCHOENLEIN PURPURA

Henoch-Schoenlein purpura (HSP) is a generalized anaphylactoid ''leuco cytoclastic'' vasculitis, which in more than 50% of patients is compli cated by gastrointestinal symptoms such as vomiting, abdominal pain, m elena and life-threatening bleedings. In five consecutive patients wit h HSP on admission factor XIII (F XIII) was decreased to 8-58% of norm al. In acute attacks an enormous F XIII consumption takes place. Recur rent release of elastase from inflammatory cells (PMNL, monocytes, mac rophages) can be detected by intermittent peaks of elastase-alpha(1)-p rotease inhibitor (ELP-alpha(1)-PI) complex in plasma (peak values 450 -950 ng/ml). Elastase is considered to cause specific F XIII degradati on with bleeding into the intestinal wall as a result. High-dose F XII I substitution therapy is effective in controlling bleeding complicati ons and abdominal pain. The benefit of F XIII therapy could be demonst rated in a rapid and marked shrinking of the intramural hematomas by u ltrasonic scan.