Henoch-Schönlein Purpura with Severe Gastrointestinal Involvement in Adult ICU Patients – Role of Blood Coagulation Factor XIII

Abstract

<i>Objective: </i>Investigation of the role of factor XIII (FXIII) and FXIII substitution in Henoch-Schönlein purpura with severe gastrointestinal involvement in adult intensive care unit patients. Design: Prospective study. Setting: Medical intensive care unit of a university hospital. <i>Patients: </i>All consecutive adult patients who were admitted to our 7-bed medical intensive care unit due to Henoch-Schönlein purpura with severe gastrointestinal involvement during a 36-month period. <i>Interventions: </i>Substitution of FXIII (Fibrogammin HS; Behringwerke, Marburg, Germany) in cases of FXIII activity being below 60% of normal range on admission. Repeated substitution until FXIII activity was increased to normal ranges and cessation of abdominal pain and bleeding was achieved. <i>Results: </i>5 patients were enrolled in the study. All had decreased FXIII activity (8-58% of normal) on admission, and in all patients FXIII substitution therapy was applied (total dose 2,500-33,750 IU). High-dose FXIII substitution &(> 15,000 IU; 4 patients) resulted in rapid cessation (1-5 days) of abdominal pain and bleeding complications accompanied by ultrasonographically demonstrable shrinking of the initially monstrous swelling of the gut wall. In the patient receiving only 2,500 IU FXIII, FXIII activity failed to increase to normal range and clinical symptoms did not improve. In all patients, intermittent peaks of the elastase-αl-protease inhibitor complex in plasma (peak values 450-950 ng/ml) were found, suggesting a recurrent release of elastase from inflammatory cells. Elastase is considered to cause a specific degradation of FXIII, leading to bleeding into the intestinal wall. <i>Conclusions: </i>In Henoch-Schönlein purpura with gastrointestinal involvement, careful and frequent examinations of the coagulation system, especially of FXIII, are necessary. The results of the coagulation data suggest that severe FXIII deficiency is due to a specific degradation by proteolytic enzymes (such as elastase) as well as consumption within the immense hemorrhagic edema of the intestinal wall. High-dose FXIII substitution therapy should be taken into consideration in cases with Henoch-Schönlein purpura and severe gastrointestinal involvement.