Today we had an excellent discussion about a 61 yo Female who presented with an acute onset of anasarca and nephrotic range proteinuria with a normal serum creatinine.
There are three major causes of nephrotic syndrome:
1) Minimal Change Disease
- Epidemiology: Most common in children, but has bimodal distribution.
- Clinical presentation: sudden onset (days to weeks) of nephrotic syndrome. Can also have microscopic hematuria. Usually have normal serum creatinine. In elderly, often present with hypertension.
- Etiology:
- Idiopathic
- Drugs: NSAIDS (most common), antibicrobials (ampicillin, rifampicin, cephalosporins), Lithium, Penicillamine, sulfasalazine, Trimethadione, gamma interferon
- Neoplasms: Hodgkin's lymphoma, non-Hodgkin's lymphoma, leukemia
- Diagnosis:
- Given the high prevalence of MCD in children, a diagnosis can often be made without a renal biopsy.
- In adults, often a renal biopsy is needed to distinguish from other causes of nephrotic syndrome.
- Treatment:
- Very steroid responsive, 80-90% achieve complete remission. Takes longer in adults than children. up to 75% respond within 6months.
- Relapses are harder to treat, and may require cyclosporin.
- In conjuction with: Low salt diet, diuretic, ACEi
2) Focal Sclerosing Glomerular Sclerosis:
- Clinical Presentation: Gradual onset of nephrotic syndrome, can have microscopic hematuria
- Etiology:
- Primary FSGS: idiopathic cause of nephrotic syndrome (mediated through circulating permeability factors, increasing the permeability of the glomeruli to albumin).
- Secondary: Viruses (HIV, Parvovirus B19, hepatitis C, EBV, CMV), drugs (IV pamidronate, anabolic steroids, interferon), obesity, lupus, reflux nephropathy, hypertensive nephrosclerosis.
- Diagnosis:
- Biopsy shows Segmental areas of mesangial collapse and sclerosis. Collapsing FSGS (HIV, iv pamidronate) characterized by collapse and sclerosis of entire glomerular tuft.
- Treatment:
- Responds to steroids, but is less responsive to steroids than MCD. For steroid resistant or relapsing disease, cyclosporine is commonly used.
3) Membranous Disease:
- Clinical Presentation: Gradual onset of nephrotic syndrome, can have microscopic hematuria
- Etiology: Solid organ tumours, lupus - class V lupus nephritis (does not need to be accompanied by other disease manifestations of lupus), infections (Hepatitis B, syphilis), Drugs (penicillamine, gold, NSAIDS)
- Diagnosis:
- ANA, C3/4, Hepatitis serology,
- Biopsy shows: Thickening of the glomerular basement membrane, in absence of hypercellularity.
- Treatment
- Control BP, ACEi, diuretic
- Prednisone +/- cyclophosphamide or cyclosporin
For more information refer to the following great articles on Nephrotic syndrome:
1 comment :
Unbelievable, I remembered I was once here in need of help on how to go about the problem of Nephrosis I was having, I must say those years was so frustrating, the experience was something outside the world of being normal,my wife had to join me in search of various kind of help,and the search lasted for 1 year and some months and like a dove sent from heaven, a friend of mine referred me to Dr Johnson a specialist with a difference full of knowledge, with his vaccines/medications and his advice on what to stay clear off and how to approach the problem, after one month of using his product, I was made well, I never believed there is a cure to Nephrosis, but now am a testimony.
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