The Role of Factor XIII and Elastase in Gastrointestinal Complications in Henoch-SchoenleinPurpura

Abstract

Henoch-Schoenlein purpura (HSP) is a generalized anaphylactoid "teucocytoclastic" 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-α 1-protease inhibitor (ELP-α1-PI) complex in plasma (peak values 450-950 ng/mi). 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 raltrascanic scan. Key Words: Henoch-Schoenlein purpura-Factor XIII—Elastase.