I would like to know if any specific immune test was done on kidney tissue. The disease represents a rare but distinct clinico-pathologic entity characterized by massive accumulation of collagen fibers in glomeruli, predominantly in subendothelial zones and the mesangium. 30 nm in diameter, curved and variably bundled, with a periodicity typical of collagen. The collagen fiber deposition if reminiscent of that seen in the nail-patella syndrome but none of the clinical or radiological features of that disorder are present. By light microscopy the glomeruli appear enlarged with an expanded, pale mesangium, minimal cellular proliferation and slightly increased lobulation.
Immunofluorescence is generally negative.
Collagen fibers are only rarely seen in glomeruli in other disorders, even in the setting of extensive sclerosis. This case was identified by the contributor as collagen glomerulopathy. Three of the other four panelists agreed. One thought it was Nail patella syndrome. I have provided below an abbreviated summary of the handout Dr. Fidler provided on the case.