The course of disease of a patient with membranoproliferative glomerulonephritis and partial lipodystrophy is described. The case is further characterized by a deficiency of C3 and C3- activator, by normal values of C4, by evidence of the nephritogenic factor, by raised fibrin degradation products and by an unselective proteinuria. The course of the glomerulonephritis runs parallel to a pronounced susceptibility to infection (at first varicella, tonsillitis and measles, later pneumonia, meningitis, encephalitis and hepatitis). On account of a nephrotic syndrome and an initative impairment of the renal function, a cytostatic treatment was begun, which although raising the C3 level did not influence the further course of the disease. As the patient has a healthy identical twin sister without lipodystrophy, who shows no reduction in C3 and no nephritogenic factor, this case proves that these diseases are acquired and not genetically determined.
Morphometric investigations in 33 patients suffering from membranoproliferative glomerulonephritis at different grades of glomerular involvement showed that there is no certain relationship between the severity of glomerular lesions and the serum creatinine level. On the other hand there is a significant positive correlation between the relative interstitial volume and the level of serum creatinine. The best congruence (r=+0,87, p less than 0.0001) showed an exponential function y=0.563-e0.049x. We therefore conclude that in membranoproliferative glomerulonephritis which is generally considered to be a glomerular disease, interstitial changes have to be taken into account as a cause of renal insufficiency. Glomerular lesions alone cannot explain the functional impairment. The pathophysiologic factors concerning increased interstitial volume as a cause of renal insufficiency - probably immunologic in origin - are yet to be completely clarified.
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