THIN GLOMERULAR BASEMENT MEMBRANE DISEASE
 
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THIN GLOMERULAR BASEMENT MEMBRANE DISEASE

 

DEFINITION:

A pathologic entity with multiple clinical expressions, including familial and sporadic forms, with either benign or progressive course.

EPIDEMIOLOGY:

  • incidence: 20% of those referred with asymptomatic isolated hematuria
  • age of onset:
    • childhood -> adult
  • risk factors:
    • familial - autosomal dominant and autosomal recessive forms
    • sporadic

PATHOGENESIS:

1. Background

  • results in a secondary glomerulopathy
  • unknown pathogenesis affecting the basement membrane (BM)

CLINICAL FEATURES:

1. Renal Manifestations

1. Hematuria

  • microscopic hematuria - persistent or intermittent
  • episodic gross hematuria associated with upper respiratory tract infections

2. Proteinuria

  • unusual but an ominous finding
  • may be associated with gross hematuria, hypertension, or renal insufficiency

3. Complications

  • Nephritic Syndrome (edema, hypertension, azotemia, oliguria)
  • Chronic Renal Failure

2. Other Manifestations

  • may be associated with some sensorineural hearing loss

INVESTIGATIONS:

1. Urinalysis

  • hematuria, proteinuria
  • microscope - glomerular pattern - damaged RBC's & RBC casts

2. Renal Biopsy

  • LM - normal
  • EM - glomerular BM thinning (not pathognomonic)
    • no areas of thickening or splitting
  • IFM - usually negative but may have glomerular deposits of immunoglobulins and complement

MANAGEMENT:

1. Supportive

  • no treatment for underlying disorder
  • multidisciplinary approach
    • Paediatrics, Nephrology, Audiology
    • long term follow-up to look for complications - proteinuria, hypertension, and renal function

2. Medical

  • medical management of complications:

1. Nephritic Syndrome

  • fluid restriction, control hypertension, manage hyperkalemia, manage pulmonary edema

2. Chronic Renal Failure

  • dialysis, renal transplantation

3. Prognosis

  • up to 40% can progress to chronic renal insufficiency and 30% to end stage renal failure

 

 

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