Current Knowledge on the Pathogenic Mechanisms of Henoch-Schoenlein Purpura in Children

Abstract

The Schӧnlein-Henoch vasculitis is an IgA-mediated inflammation of small vessels. The main clinical manifestation is palpable purpura without thrombocytopenia, manifested by bilateral symmetrical distribution on both lower limbs, abdominal pain, often accompanied by blood in the stools, hematuria with / or without proteinuria. Historically, the disease has been known since 1832. In Bulgaria, although that later Henoch-Schӧnlein purpura (HSP) aroused scientific interest, several publications focused on the pathogenic nature, clinical variations, and potential complications of the disease, as well as options for therapeutic intervention. Modern knowledge about the etiology and pathogenesis of the disease has given new light, respectively a basis for early diagnosis, reduction of complications and recurrences, as well as the avoidance of polypragmatism. It turned out that in HSP there are genetic factors and new aspects, binding until now accepted concept that there are no changes in the coagulation system. The role of factor XIII, as a prognostic indicator of the severity of clinical manifestations and von Willebrand factor (vWF), as a reliable index of the degree of vascular wall damage, has been demonstrated. Plasma imbalance in the Th1 / Th2 ratio was also found and a percentage of NK cells, respectively their immune function, were reduced. This new knowledge about the pathogenic nature of HSP is the basis for a change in the concept of therapeutic behavior, which is aimed at the severity of the clinical course, and not only at the treatment of the disease, which is itself symptomatic.