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