2- nephritic syndrome

    Cards (20)

    • Nephritic syndrome

      Association of hematuria with RBC casts, sub-nephrotic range proteinuria, edema, hypertension, oliguria, azotemia, and renal impairment/failure
    • Nephritic syndrome

      • Due to glomerular injury with glomerular inflammation secondary to an immunological event
      • Immune-complex deposition activates complement à C5a attracts neutrophils à damage to basement membrane, mesangium, or endothelium
    • Presentations of nephritic syndrome

      • Acute nephritic syndrome (acute glomerulonephritis)
      • Rapidly progressive glomerulonephritis
      • Chronic glomerulonephritis/chronic renal failure
    • Causes of nephritic syndrome
      • Post-streptococcal glomerulonephritis
      • SLE
      • Membranoproliferative glomerulonephritis
      • Cryoglobulinemia
      • Anti-GBM (Goodpasture syndrome)
      • IgA nephropathy (HSP)
      • Vasculitis
    • Post-streptococcal glomerulonephritis
      • A diffuse proliferative type of glomerulonephritis
      • Most common cause of acute nephritis worldwide
      • Group A strept (strept. Pyogens)
      • Occurs 1–12 weeks after a sore throat or skin infection (streptococcal antigen deposited on the glomerulus à host reaction and immune complex formation)
    • Presentation of post-streptococcal glomerulonephritis

      • Microscopic hematuria
      • Acute nephritic syndrome (gross hematuria, proteinuria, edema, hypertension, and acute kidney injury)
    • Diagnosis of post-streptococcal glomerulonephritis

      Findings of acute nephritis + demonstration of a recent GAS infection (positive throat or skin culture or serologic tests [anti-streptolysin titer ASOT), low complement, RBC casts
    • Renal biopsy findings in post-streptococcal glomerulonephritis
    • Management of post-streptococcal glomerulonephritis

      • Supportive, >95% recover normal renal function
      • Antibiotic therapy is given to those who have still have a streptococcal infection at the time of diagnosis
    • Anti-GBM (Goodpasture syndrome)
      • Caused by autoantibodies to type IV collagen, which is highly expressed in the GBM and alveoli
      • Usually presents with rapidly progressive glomerulonephritis: acute renal failure (may occur within days), nephritic urine sediment, and non-nephrotic proteinuria
      • Pulmonary involvement (alveolar hemorrhage à hemoptysis & dyspnea) is present in 40 to 60% of patients, especially in smokers
    • Diagnosis of Anti-GBM (Goodpasture syndrome)
      Demonstration of anti-GBM antibodies, so kidney biopsy is required: Linear deposits of IgG along the GBM is diagnostic
    • Treatment of Anti-GBM (Goodpasture syndrome)
      • Plasma exchange to remove the autoantibodies, steroids ± cyclophosphamide
      • If treatment is started early, full recovery is possible, and relapses are rare
      • Renal prognosis is poor if dialysis-dependent at presentation
    • IgA nephropathy (HSP)
      • Most common cause of chronic glomerulonephritis in developed countries
      • Common cause of mild recurrent hematuria
      • Most patients present with either gross hematuria (single or recurrent), usually accompanying an upper respiratory infection or after exercise, or microscopic hematuria with or without mild proteinuria incidentally detected on a routine examination
      • Occasionally they might present with rapidly progress glomerulonephritis & nephritic syndrome
      • lgA production is increased during infections à forms immune complexes and deposits in mesangial cells
      • When accompanied by extra-renal symptoms: purpuric skin lesions, abdominal pain, arthralgia à Henoch-Schoenlein purpura
    • Renal biopsy findings in IgA nephropathy
    • Treatment of IgA nephropathy
      • Supportive
      • ACEI should be given to all patients
      • Steroids/immunosuppression may slow decline of renal function if persistent proteinuria
    • Prognosis of IgA nephropathy
    • Investigations for nephritic syndrome
      • Urine microscopy for red cells and casts
      • RFT (Serum urea and creatinine raised)
      • Creatinine clearance (low)
      • 24-hour urine protein (raised)
      • C3 and C4 (low), Normal complement in IgA nephropathy & anti-GBM
      • Chest X-ray for pulmonary edema
      • Renal imaging (normal size)
      • Renal biopsy if needed
      • Diagnostic tests for specific causes: Throat swab, Antistreptolysin O titer, ANCA, Anti-GBM antibody, Cryoglobulins
    • General management of nephritic syndrome

      • Daily weight checks
      • Fluid balance chart
      • Antihypertensive & BP monitoring
      • Salt restriction
      • Fluid restriction
      • Diuretics
      • Dialysis if needed
      • Specific treatment for each cause if needed
    • Alport syndrome

      • Congenital defect of collagen IV à glomerular disease
      • Sensorineural hearing loss, visual disturbances (lens lenticonus)
      • Hematuria and hypertension
      • Always in males X-linked dominant
      • Histology: basket weave shaped
    • Thin membrane disease

      • Only hematuria
      • Very benign
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