The vast majority of people answered correctly; the best answer is D.Approximately 3-5% of patients with Alport's syndrome develop de novo anti-glomerular basement membrane (GBM) disease in the transplanted kidney. Alport’s syndrome is a genetic disorder that results from mutations in the genes encoding the alpha-3, alpha-4, or alpha-5 chains of type IV collagen. Following transplantation, recipients can become alloimmunized and develop antibodies to the normal chains of type IV collagen in the basement membrane of the donor kidney (hence, the linear pattern of staining noted on IF). Treatment is not standardized, but generally consists of plasmapharesis, +/- cyclophosphamide and steroids. Long-term allograft survival is poor and re-transplantation carries a high risk of anti-GBM recurrence. For the boards, remember the post-transplant association between Alport’s syndrome and de novo anti-GBM disease.
Link to NEJM review which contains above picture
Michael Lattanzio DO
Hi , I am a nephrology student about to appear for my boards this december . I find your blog to be extemely useful and interesting. I would request u to focus on Clinical Pearls in nephrology especially for students like me.
ReplyDeleteAlso could u enumerate other diseases that cause Linear staining on IF
Thanks, we will try and do our best. I believe that linear staining on IF is rather pathognomonic for anti-GBM disease or goodpasture's disease/syndrome.
ReplyDelete