Glomerular Disorders

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  • Nephritic syndrome
  • Rapid progressive glomerulonephritis

Nephritic Syndrome

Description and clinical presentation

  • Mild proteinuria
  • Hematuria
  • High blood pressure
  • Acute reduction in GRF
  • Some oedema

Causes
Reduced complement

  • Post streptococcal Glomerulonephritis
  • Shunt Nephritis
  • Endocarditis
  • SLE
  • HCV
  • Athero-emboli GN

Normal compliment

  • IgA
  • HSP
  • Anti-GBM
  • ANCA positive GN

Clinical features
History of sore throat especially children, features of lupus, purpura(HSP, HCV), peripheral neuropathy (HSP, HCV), pulmonary haemorrhage (ANCA), chronic sinusitis (ANCA), associated asthma (ANCA)

Diagnosis

  • ANA, ANCA, ds-DNA, ASOT, DNAse, HBsAg, AntiHCV, anti-GBM, RPR, C3, C4
  • Renal biopsy except for post-streptococcal

Therapy

  1. Post streptococcal
    • Supportive
    • BP control
    • Antibiotic
    • Fluid management
    • Dialysis when indicated
    • Prognosis good
  2. ANCA positive/Anti-GBM
    • See under Rapid Progressive Glomerulonephritis
  3. Systemic Lupus Erythematosus
    • See Lupus Nephritis
  4. Others (HBsAg, HCV, IgA, HSP, Shunt nephritis)
    • Treat cause

Rapid Progressive Glomerulonephritis

Description

  • Sub-acute reduction in renal function as opposed to acute nephritis that is rapid.
  • Takes a few weeks to few months for renal function to deteriorate

Clinical features

Similar to acute nephritis except this is more insidious, Hemoptysis, asthma, sinusitis, epistaxis, abdominal pain, peripheral neuropathy, petechiae, purpura.

Causes

  • Type 1
    • Anti-Glomerular basement disease (Anti-GBM)
  • Type 2
    • Immune complex disease
    • SLE
    • Post streptococcal
    • IgA/HSP
  • Type 3: ANCA positive
    • Polyangiitis with granulomatosis (Wegners)
    • Eosinophilic granulomatosis with polyangiitis (Churg Strauss)
    • Microscopic polyangiitis

Management

  • Serum p-ANCA and C-ANCA
  • Renal biopsy is mandatory for light, Immunoflourence, electron microscopy.\
  1. Anti-GBM RPGN
    1. Prednisolone 60 mg/day and reducing
    2. Cyclophosphamide 2mg/kg/day and adjusted for white cell count
    3. Plasma exchange (50ml/kg to a maximum of 4L daily for 14 days or until anti-GBM antibodies undetectable)
    4. Treat for 6 months
  2. Immune complex RPGN
    1. except for Streptococcal, treat as in 3 except plasma exchange may not be indicated
  3. ANCA positive RPGN
    1. Methylprednisolone 7mg/kg for 3days and then prednisolone 1mg/kg/day for 4 weeks then taper with either
    2. Cyclophosphamide 0.5g/m2 IV monthly for 6 months OR
    3. Cyclophosphamide 2mg/kg PO for 6-12 months
    4. Plasma exchange for patients with lung haemorrhage and renal dysfunction
    5. Co-trimoxazole prophylaxis.