The Nephrotic Syndrome in Congenital Syphilis: An Immunopathy

This report describes the renal morphology in an infant, 2½ months of age, who had the nephrotic syndrome secondary to congenital syphilis. A specimen of the kidney obtained by needle biopsy was studied by light, electron, and fluorescent microscopy. Periarterial cellular infiltrates composed of lymphocytes and plasma cells were prominent in the interstitial tissues. Glomerular visceral and parietal epithelial cells were swollen and increased in number. The axial matrix in many glomeruli was expanded by partially collagenized axial matrix mateial. Nodular electron dense deposits were numerous in the epithelial aspect of the glomerular basement membrane. The areas corresponding to the electrondense deposits were strongly stained with fluorescein tagged antisera to IgG, IgM, and fibrinogen. Less intense but still positive staining was obtained with antisera to IgA and β1c globulin. The nephropathy in congenital syphilis appears to have its basis in the deposition of immune complexes within the glomerular basement membrane. The very young infant, therefore, appears capable of manifesting with an immune deposit disease. The presence of all classes of immunoglobulins in the membranous deposits suggests that the infant has precocious capabilities for producing each of these immunoglobulins.