Nephritic Syndrome: Goodpasture's Syndrome = CRESENTERIC GN

Question 1

Definition

Answer 1

Autoimmune disease that primarily lungs and the kidneys causing pulmonary haemorrhage and glomerulonephritis.

Question 2

Epidemilogy + Risk Factors

Answer 2

Rare in children
+ 16
Men
HLA-DR15
Smoking
Infection
Oxidative stress
Hydrocarbon-based solvents

Question 3

Pathophysiology

Answer 3

Type 2 hypersensitivity reaction = autoantibodies usually IgG but rarely IgM or IgA bind to the a3 chain of a Type 4 collagen = activate the complement system.
C3a, C4a, and C5a act as chemotactic agents meaning they attract certain cells like neutrophils.
Once neutrophils are involved they release enzymes like peroxidase, myeloperoxidase, and proteinase-3 which all cause free oxygen radicals to form which damage the basement membrane as well as the nearby endothelium and the underlying organ itself.

Question 4

Signs

Answer 4

Lung symptoms before kidney symptoms
- haemoptysis
- restrictive lung disease
- cough
- SOB
- Pulmonary haemorrhage
Kidney
- haematuria
- proteinuria
- oliguria
- HTN
Constitutional symptoms:
- lethargy
- fever
- anorexia
- weight loss
- myalgia
- arthralgia

Question 5

Diagnosis

Answer 5

Kidney biopsy
- Under a microscope = inflammation of the basement membrane = CRESCENTIC glomerulonephritis
- fluorescence = linear deposition of IgG along glomerular capillaries
Autoantibodies = anti-GBM

Question 6

Treatment

Answer 6

Corticosteroids + immunosuppressants (cyclophosphamide) + plasmapheresis
Bronchoscopy or angiography + embolism = to control pulmonary haemorrhage