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31 Cards in this Set
- Front
- Back
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Nephrotic Syndrome
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Proteinuria > 3.5 g/day/1.73 m2
Hypoalbuminemia < 3.5 g/dL Edema Hyperlipidemia Lipiduria unless low albumin due to chronic nephrotic syndrome |
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Causes of primary idiopathic NS
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Minimal change disease
Membranous nephropathy Focal segmental glomerulosclerosis Membranoproliferative glomerulonephritis IGA nephropathy |
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Causes of secondary NS
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Minimal change disease
Membranous nephropathy Focal segmental glomerulosclerosis Membranoproliferative glomerulonephritis Diabetic nephropathy Amyloid Light change deposition disease (multiple myeloma) |
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Minimal Change Disease
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Most cases primary/idiopathic
Secondary causes Nonsteroidal anti-inflammatory agents Malignancies (hematologic) Presents with proteinuria, edema, hypoalbuminemia, hyperlipidemia. Hematuria, hypertension, and renal failure are uncommon. Light and immunofluorescence reveal no abnormalities Electron microscopy reveals effacement of the foot processes |
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Minimal Change Disease- Treatment
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Very responsive to steroid therapy
90% of children with nephrotic syndrome have steroid responsive minimal change disease and generally do not require biopsy unless they do not respond to therapy Primary etiology unclear Loss of negative charge at membrane allows for protein leakage |
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Focal Segmental Glomerulosclerosis
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Number one cause of the nephrotic syndrome in adults
More common in African Americans Also frequently secondary to systemic diseases such as HIV Can recur in renal transplant Nephrotic syndrome with benign urinary sediment |
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FSGS- Therapy
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Prednisone for up to 6-9 months.
Immunosuppressants with cyclosporine, mmf, and possibly cytoxan Ace inhibition or ARB agents Poor prognosis in patients with high grade proteinuria, fibrosis, and renal failure at presentation |
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Secondary causes
of FSGS |
Healing of previous glomerular injury
Massive obesity ? OSA Sickle cell anemia HIV (other viruses) Pamidronate Heroin abuse Treat the underlying disease or stop the offending agents. |
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Membranous Nephropathy
Secondary causes |
Malignancy, primarily solid tumors
Class V lupus nephritis Rheumatoid arthritis Hepatitis B and C Drugs (penicillamine, gold, NSAID’s, captopril) Syphilis |
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Membranous Nephropathy
courses |
Primary membranous nephropathy now the second most common cause of the nephrotic syndrome in adults
Very variable course 30% with spontaneous remission 30% with persistent proteinuria 30% with progressive renal failure Important to try and predict who will progress |
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membranous nephropathy risk factors
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Risk factors for progressive renal disease somewhat controversial
Some studies suggest male sex, age over 50 as risk factors Most studies indicate patients with renal failure, persistent nephrotic syndrome, should be treated with immunosuppressant therapy Patients with high grade proteinuria and hypoalbuminemia are at high risk for thrombotic events, require anticoagulation Patients in high risk group are generally treated with steroids and either cyclophosphamide or chlorambucil. Newer therapies utilizing MMF or rituxan still in earlier phases of investigation Acth also utilized in small study |
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Treatment Algorithm for Membranous Nephropathy
draw table |
oxman ppt 21 lecture 5
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Secondary Renal Diseases and the Nephrotic Syndrome
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Diabetes Mellitus
Systemic Amyloidosis Multiple Myeloma Lupus |
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Diabetes Mellitus
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Single most common cause of ESRD in the United States
Type I Diabetics with nephropathy have 50 fold increase mortality compared to those without nephropathy Type I Diabetic nephropathy generally occurs 10-20 years from disease onset Develop nodular glomerulosclerosis Urinalysis generally without cells Occasional hematuria but if present need workup for other causes of hematuria Proteinuria starts mild but can progress to nephrotic syndrome Treatment with BP control and utilizing ACE/ARB with possible use of newer renin inhibitors |
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Diabetes Mellitus
staging |
Stage I- increased GFR , reduces with initiation of glucose control
Stage II- increased GFR, hyperfiltration, GBM thickening noted Stage III- microalbuminuria 30-300/day. Harbinger for future nephropathy. Frequently hypertensive as well Stage IV- dipstick positive proteinuria. Decline in GFR 0.5-1.0 ml month. Can be altered by antihypertensive therapy. Stage V- ESRD |
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Diabetes Mellitus type 1-2 diff and staging
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Five stages generally noted in Type I Diabetes but similar in Type II
More difficult to date the time of onset of Diabetes in the Type II patient Retinopathy present in nearly 100% of Type I Diabetics with nephropathy, 2/3 of patients with Type II Diabetes and nephropathy |
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Systemic Amyloidosis
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90% of patients have renal involvement; 60% have nephrotic syndrome
Diffuse hyaline material deposited in glomeruli Confirmed by special stains- Congo red Diagnosis made via rectal, abdominal fat pad, skin and renal biopsy Primary amyloid affects the heart, kidney, and nerves 90% have paraprotein detected in serum Mean survival is 2 years Mephalan and prednisone Bone marrow or stem cell transplant Renal failure portends a poor prognosis |
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Secondary Amyloid
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Chronic inflammatory states- RA, IBD, some malignancies renal cell CA
Treat underlying disease |
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Fibrillary GN and Immunotactoid GN
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Diagnosed by renal biopsy
Different sized fibrils noted Patients generally present with nephrotic syndrome No effective therapy currently |
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Monoclonal Deposition Diseases
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Light chains do not form fibrils
Kappa light chain frequently detected in serum or urine by immunofixation Multiple myeloma and cast nephropathy can be seen Renal dysfunction in these patients portends a poor prognosis Treat underlying disease? Plasmapharesis |
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Lupus Nephritis- nephrotic syndrome class
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CLASS 5
Occurs in half the patients with lupus Class V lupus nephritis presents with nephrotic syndrome Most other forms of lupus present with nephritic syndrome |
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Treatment of Nephrotic Syndrome Complications
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In addition to treating the underlying disease also need to treat systemic manifestations of the disease.
Edema Hypoalbuminemia Hypercoagulability Hyperlipidemia Drug Binding Infection |
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Edema
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Low Sodium Diet
Loop Diuretics Start with low dose and then double dose to obtain response Add Thiazide diuretics such as metalozone May occasionally need IV diuretics Little role for albumin but occasionally used Goal is for 1-2 pounds of fluid loss/day |
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Edema- Diuretic Resistance
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Patients can develop diuretic resistance
Related to excess sodium intake Antagonistic drugs ie NSAIDS Inadequate dosing Poor oral absorption |
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Hypoalbuminemia
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Insure adequate nutrition; no high protein diet
Use ACE/ARB, non-dihydropyrodine calcium channel blockers to reduce protein excretion Adequate BP control Treat underlying disorder |
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Hypercoagulability
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Increased coagulation tendency
Low antithrombin 3, loss of clotting inhibitors in urine, decreased fibrinolysis Enhanced platelet aggregation Risk for DVT, RVT, PE Membranous nephropathy with greatest risk up to 35% |
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Renal Vein Thrombosis
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10% of nephrotic patients; 35% membranous
Frequently asymptomatic Can present with renal failure if bilateral May present with flank pain, hematuria Pulmonary embolism is life threatening complication |
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Anticoagulation
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Selective approach, individualize per patient
Reasonable prophylactic anticoagulation in patients with patients with severe nephrotic syndrome with albumin less than 2.2 INR 1.8-2.0 Continue treatment while nephrotic Watch for thrombotic events |
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Hyperlipidemia
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Generally accepted that nephrotic patients have five fold increase in cardiac events
Hyperlipidemia may contribute to progressive renal disease Increased hepatic synthesis of LDL and VLDL Will need to treat underlying disease Select high risk patient Use ACE/ARB agents to reduce protein excretion Treat underlying disease Dietary therapy Utilize statins Clofibrate dose must be modified as it can induce severe myopathy in patients with nephrotic syndrome |
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Infection
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Nephrotic patients may be prone to bacterial infection
Pneumocoocal peritonitis seen in nephrotic children Loss of IgG and complements may inhibit ability to clear encapsulated organisms |
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Conclusions- main point
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Multiple causes of the nephrotic syndrome
Diabetes the most common secondary cause FSGS most common cause in adults; minimal change in children Treat all patients with diet, blood pressure control Proteinuria a marker for underlying renal and CV disease Smoking Cessation |
