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Q J Med 1990; 75: 387-398
© 1990 Association of Physicians

other

Familial Incidence of C3 Nephritic Factor, Partial Lipodystrophy and Membranoproliferative Glomerulonephritis

DAVID A. POWER*, YIN C. NG**,** and JOHN G. SIMPSON{dagger}

*Department of Medicine & Therapeutics University of Aberdeen Foresterhill, Aberdeen AB9 2ZD {dagger}Department of Pathology, University of Aberdeen Foresterhill, Aberdeen AB9 2ZD **MRC Clinical Immunology Group, Department of Medicine, Royal Postgraduate Medical School, Hammersmith Hospital Ducane Road, London W12 0NN

Accepted for publication 7 July 1989.

C3 nephritic factor is an IgG autoantibody that causes complement activation by stabilizing the alternative pathway C3 convertase. It is associated with partial lipodystrophy and membranoproliferative glomerulonephritis. The occurrence of C3 nephritic factor, partial lipodystrophy and membranoproliferative glomerulonepharitis, whether singly or in any combination, is usually sporadic. We describe the coexistence of all three of these conditions in members spanning two generations of a single family. This suggests that the pathogenesis of these conditions may be linked and that genetically determined factors may, in some circumstances, contribute to disease susceptibility.

**Current address: Division of Molecular & Clinical Rheumatology, The Johns Hopkins University School of Medicine, Baltimore MD 21205 USA


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