Lecture 14: Goodpasture's Disease

Question 1

What is Goodpasture’s disease?

Answer 1

Also known as anti-glomerular basement membrane antibody (anti-GBM) disease.

It is an important cause of pulmonary-renal syndrome.

60-80% of patients have pulmonary and renal disease.
20-40% have renal disease alone
>10% have disease that is limited to the lungs.

Caused by an autoantibody to the alpha-3 chain of type IV collagen.

A specific human leukocyte antigen type (HLA-DRB1*1501 or HLA-DR4) is considered genetic marker.

Question 2

Describe the epidemiology of Goodpasture’s.

Answer 2

It is quite rare, with an estimated incidence of 1 to 2 cases per million.

It is responsible for 1-2% of all cases of glomerulonephritis.

30-50% of patients will also have anti-neutrophil positive antibodies.

Question 3

How can Goodpasture’s disease be diagnosed?

Answer 3

Symptoms may be minimal or include cough, SOB, haemoptysis fatigue, malaise, dark urine, decreased urine output and leg oedema.

Question 4

What Anti-Neutrophil Cytoplasmic Antibodies (ANCA) can be tested for in Goodpasture’s?

Answer 4

p-ANCA and c-ANCA.

p-ANCA, also known as anti-myeloperoxidase antibody.

c-ANCA, also known as anti-proteinase-3 antibody.