Goodpasture's syndrome Flashcards
Goodpasture’s syndrome is a type of neph____ disease
nephritic
What is another name for Goodpasture’s syndrome?
Anti-glomerular basement membrane (anti-GBM) disease
Goodpasture’s syndrome is a rare _____ disease characterised by antibodies targeting the basement membrane of kidneys and lungs.
autoimmune
Goodpasture’s syndrome is a type _ hypersensitivity reaction
2
Goodpasture’s is damage of the b_____ m___ in the l___ and k_____
basement membrane, lungs and kidneys
What type of collagen is a major scaffold structure underlying glomerular basement membrane?
Type 4 collagen
Ig_ antibodies bind to alpha 3 folded chain which activates the complement system and leads to damage of the collagen fibres of the basement membrane.
IgG
Goodpasture’s syndrome can rapidly progress to g____ and potentially life-threatening lung haemorrhage
glomerulonephritis
What are symptoms of Goodpasture’s syndrome?
Haematuria
Proteinuria
Decline in kidney function
Cough
SOB
Haemoptysis
Pulmonary haemorrhage
True or false: pulmonary manifestations usually occur before renal ones
True
eg Cough, haemoptysis, dyspnoea first from damaged alveoli
What are risk factors for Goodpasture’s syndrome?
Bimodal distribution: 20-30 males and 60-70 females
Genetic predisposition for genes that code for HLA-DR15
Environmental: infection, smoking, oxidative stress, hydrocarbon-based solvents
How do you diagnose Goodpasture’s syndrome?
Renal biopsy:
Anti-GBM antibodies
Crescentic glomerulonephritis
Electron microscopy showing thickened glomerular basement membrane
May have elevated creatinine and urea
Chest CT or XR may show pulmonary haemorrhage
What is crescentic glomerulonephritis?
The pattern of glomerular injury , crescent-shaped formation in the glomeruli composed of proliferating cells and infiltrating inflammatory cells
What is the main type of cell proliferating in crescentic glomerulonephritis?
Parietal epithelial cells
What are complications of Goodpasture’s syndrome?
Chronic renal failure requiring dialysis
