If appropriate treatment is not provided, a significant portion of patients with primary FSGS will progress to ESRD. Only a small minority of patients experience complete spontaneous remission. Patients who progress to end-stage renal disease and receive a renal transplant are at risk for recurrent FSGS in the allograft.

C1Q NEPHROPATHY

C1q nephropathy is a rare but distinct cause of nephrotic syndrome with a clinical presentation and histopathologic findings that are very similar to what is seen with FSGS. Like FSGS, C1q nephropathy is also more common among African Americans. The pathogenesis is unknown but is likely immune-mediated. The major feature that distinguishes C1q nephropathy from FSGS is the presence of mesangial immune complex deposits that stain positive for C1q on immunofluorescence. Staining is often positive for IgG, IgM, and C3 as well. There is no established treatment for C1q nephropathy. Some clinicians advise steroids, although the literature has offered mixed results regarding its efficacy.