Abstract

C1q nephropathy (C1qNP) is a controversial and uncommon form of glomerulonephritis characterized by mesangial immunoglobulin and complement deposits, predominantly C1q, with no evidence of systemic lupus erythematosus. Clinically it may present as nephrotic syndrome and non-nephrotic proteinuria per se or associated with microhematuria, hypertension, or renal insufficiency.

We describe two sisters with C1qNP, who presented with steroid resistant nephrotic syndrome. Both sisters presented before the age of 2 years and they showed a poor response to other immunosuppressive therapy as well. Both girls had normal serum complement levels, negative antinuclear antibodies (ANA) and negative hepatitis B antigen. Renal biopsy in both patients showed histological features of mesangioproliferative glomerulonephritis with diffuse full house positive immunofluoresence reaction in the mesangial area. The immunofluorescent reaction for C1q was the most intense and co-dominant with IgG in both patients. Correspondingly, electron microscopy demonstrated dense deposits mainly in the mesangial areas too.

We report two young sisters with the characteristic features of C1qNP presented in early childhood. To the best of our knowledge this is the first report of C1qNP in siblings.