Introduction Secondary nephrotic syndrome is a multisystem disease primary nephrotic syndrome refers to disease limited to the kidney Presentation is similar to primary nephrotic syndrome Snapshot A 69-year-old male with a past medical history of hypertension, dyslipidemia, obesity, and type II diabetes mellitus presents for a general checkup. On fundoscopic exam, retinal hemorrhages and hard exudates are seen. The patient's blood pressure is 175/100 mmHg. As the visit continues the patient admits to not taking his insulin regularly and has eliminated fruits and vegetables from his diet. The patient is promptly started on lisinopril. Diabetic Glomerulonephropathy Most common cause of end stage renal disease in USA caused by poor glycemic control severity of renal disease is highly correlated with severity of diabetic retinopathy Caused by nonenzymatic glycosylation of glomerular basement membrane (GBM) results in ↑ permeability and thickening Hyaline arteriolosclerosis of efferent arterioles also present results in ↑ GFR and hyperfiltration injury eventually the afferent arteriole also hyalinizes which ↓ GFR Early manifestation is microalbuminuria detected by special microalbumin dipsticks Light microscopy shows pathognomonic Kimmelstiel-Wilson nodules, thickened GBM, and mesangial expansion Treatment can slow progression with ACE inhibitors strict glycemic and hypertension control transplant is only treatment after disease has progressed Snapshot An African-American, HIV postive, IV drug using male presents for a routine check-up. The patient admits to not being compliant with his medications. Lab work is ordered and demonstrates a CD4+ count of < 50. A urine dipstick is positive for 2+ red blood cells and 1+ protein. The patient is sent to a nephrologist and ultimately a biopsy is performed and is seen to the right. HIV Glomerulonephropathies Usually seen in HIV acquired through IV drug use Presents with focal segmental glomerulonephritis Light microscopy shows segmental sclerosis and hyalinosis Treat HIV Snapshot A young female presents for a regular check-up. She complains of a rash that she gets on her face in a raccoon distribution when she is out in the sun. She also states that she has joint pain as well as some chest pain that is relieved when she leans forward. Given the clinician's suspicion, ANA and anti-dsDNA are ordered and come back positive. A urine dipstick is performed demonstrating 2+ proteinuria and 3+ red blood cells. Lupus Glomerulonephritis Type I minimal change in glomerluar structure Type II mesangial disease with focal segmental glomerular pattern Type III focal proliferative disease treat with aggressive prednisone +/- cyclophosphamide Type IV most severe form diffuse proliferative disease presents as combination of nephritic/nephrotic disease classic wire loop abnormality treat with prednisone, cyclophosphamide, mycophenolate, and transplant Type V membranous disease indistinguishable from other primary membranous glomerulonephropathies treatment: consider prednisone Snapshot A 69-year-old man presents to your office with back pain that has been going on for, "quite some time." He also states he has felt lethargic and not quite himself. Lab values are ordered demonstrating an elevated serum calcium. A urinalysis demonstrates monoclonal antibodies in the urine (Bence-Jones proteins) and a peripheral smear is performed and seen to the right. Amyloidosis Two types primary amyloidosis AL amyloid present primarily seen in multiple myeloma secondary amyloidosis AA amyloid present primarily seen in conditions of chronic inflammation (e.g., TB and rheumatoid arthritis) Light microscopy show amyloid with congo red stain apple-green birefringence with polarized light