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Key Features

  • Relatively rare pattern of glomerular injury

  • Can be caused by wide range of identifiable etiologies or can be idiopathic

  • Can present anywhere along the spectrum of nephritides, ranging from asymptomatic glomerular hematuria to acute nephritic syndrome with bouts of gross hematuria to rapidly progressive glomerulonephritis (RPGN)

  • Type I is relatively more common and can be idiopathic (especially in children and young adults) or secondary to a chronic infection (most commonly hepatitis C virus), a paraproteinemia, or an underlying autoimmune disease such as systemic lupus erythematosus

  • Type II is caused by several inherited or acquired abnormalities in the alternative complement pathway

  • The two types are primarily distinguished by immunofluorescence and electron microscopy

Clinical Findings

  • Low circulating C3 complement

Diagnosis

Type I

  • Low C4 complement

  • Chronic antigenemia leading to classic complement pathway activation with immune complex deposition

  • Kidney biopsy

    • Light microscopy shows mesangial hypercellularity, endocapillary proliferation and capillary wall remodeling resulting in double contours of the glomerular basement membrane ("tram track" appearance)

    • Electron microscopy shows scattered subendothelial and subendothelial deposits

    • Immunofluorescence shows positive immunoglobulin and C3 staining

Type II

  • Kidney biopsy

    • Light microscopy findings similar to type I

    • However, electron microscopy shows thick ribbon-like electron dense deposits along the glomerular basement membrane

Treatment

  • Treatment is controversial

  • For mild disease, angiotensin-converting enzyme inhibitors and angiotensin receptor blockers should be used

  • For severe disease

    • A combination of oral cyclophosphamide or mycophenolate mofetil plus corticosteroids could be considered

    • Rituximab is also sometimes used

  • Patients with RPGN and crescents on biopsy may be treated as for ANCA-associated disease provided secondary causes have been ruled out

  • Despite therapy, end-stage renal disease will develop in most patients

  • Small, uncontrolled series suggest a possible benefit of eculizumab

  • Prognosis less favorable with type II disease, early decline in glomerular filtration rate, hypertension, and persistent nephrotic syndrome

  • Kidney transplantation may be undertaken, but both types may recur afterwards

  • Plasma exchange has been used with mixed results to treat post-transplant recurrence of membranoproliferative glomerulonephritis

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