743 UTILIZATION OF PROPERDIN IN CHILDREN WITH VASCULITIS AND MILK ALLERGY

Abstract

Patients with various types of vasculitis have serologic findings which indicate that properdin is consumed during disease activity. In all of 22 patients with documented Henoch Schönlein Purpura, the level of serum properdin was reduced more than 3 standard deviations below the mean in the first week of the disease. Patients with predominant signs of abdominal pain and glomerulonephritis had more pronounced reductions in serum properdin than did those patients with arthritis as a presenting symptom. Serum levels of properdin convertase were also reduced in 12 of the 22 patients. As the disease subsides, levels of properdin and properdin convertase return to normal. All other serum complement components and alternative pathway proteins were normal. Similarly, six patients with nonspecific vasculitis had isolated reductions in serum properdin which correlated well with disease activity. Of great interest is the fact that 3 patients with milk allergy also had isolated low levels of serum properdin. The level of properdin returned to normal on a milk-free diet and fell on challenge with milk. In addition, these latter patients demonstrate a functional inadequacy of the alternative pathway as judged by incomplete C3-C9 consumption on the addition of zymosan to their serum. These findings suggest that properdin and properdin convertase may be involved in those disorders effecting vascular inflammation.

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