Serum Complement Defects in Periodic Diseases

After Donaldson and Evans, and Austen and Sheffer demonstrated a deficient serum inhibitor of C1 esterase during periodic edema (hereditary angioneurotic edema), study of our patients with periodic polyserositis, arthrosis, myelodysplasia, and fever usually showed deficiency of the inhibitor and of whole serum complement in some. The inhibitor and complement activity were lowest in periodic arthrosis, especially during episodes of the disease. This and other features in common further indicate a relationship of these disparate heritable disorders. Demonstration of the lack of the inhibitor and of whole serum complement aids in diagnosis. It is uncertain if the defects are causal, incidental, or consequences of the diseases and if correction of the deficiencies will relieve symptoms.