Sequential Measurements of the Reticulo‐Endothelial System Function in Henoch‐Schönlein Disease of Childhood: Correlations with Various Immunological Parameters

Abstract

Different immunological parameters were studied in 16 children suffering from Henoch-Schönlein purpura. The following results were observed during the acute phases in some patients: (1) an increase in C3d plasma levels; (2) the presence of circulating immune complexes (CIC); (3) an increase in IgA plasma levels and (4) an impairment of the reticuloendothelial system (RES) function assessed by an in vitro and an in vivo test. After the acute phase, all the altered parameters were almost normalized in recovering patients. On the contrary, all 5 patients with persistent urinary findings or relapsing purpura continued to present increased IgA plasma levels and/or CIC and/or impaired RES function. Our results therefore show that, in Henoch-Schönlein disease of childhood, a correlation exists between persisting clinical signs and persisting high IgA plasma levels, CIC and RES function impairment.

Berger disease: thirty years later

“…However, the poorly galactosylated IgA found in IgAN [2,21] is possibly cleared to a lesser extent than normal IgA by the hepatocyte receptor for asialoglycoproteins. In contrast to the clearance mechanisms of normal IgA, a reduction of Fc gamma, C3b and ®bronectin receptor functions of mononuclear phagocytes has been reported in IgAN [22,65]. Such abnormalities are usually not correlated with clinical signs; they are transient and seem to result from the saturation or receptors rather than being the primary event.…”

Serum IgA/C3 Ratio May Be a Useful Marker of Disease Activity in Severe Henoch-Schönlein Nephritis

“…However, an activation of the complement occurs since degradation products have been demonstrated in plasma and glomeruli [20, 22]. However, the role of this activation remains unclear.…”

“…Although the levels of serum C3 tend to decrease in the active stage of chronic glomerulonephritis, those are not generally decreased in HSN [20, 21]. However, an activation of the complement occurs since degradation products have been demonstrated in plasma and glomeruli [20, 22].…”

Severe gastrointestinal vasculitis in Henoch-Schoenlein purpura: pathophysiologic mechanisms, the diagnostic value of factor XIII, and therapeutic options

“…Therefore, they speculated that factor XIII deficiency in rats with bacteremia might contribute to the elevation in plasma fibronectin by altering kinetics associated with the clearance of fibronectin from the blood [8]. Because the function of the reticuloendothelial system is impaired in the acute stage of HSP [2], and the level of IgA-fibronectin aggregates are also elevated in the serum of patients with HSP [5], decreased factor XIII activity might have influenced severe gastrointestinal hemorrhage in HSP.…”