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Goodpasture’s Syndrome

Title               

Ñ    Goodpasture’s Syndrome

Definition

Disease characterised by the following:

Ñ    Lung haemorrhage

Ñ    Glomerulonephritis

Ñ    Anti-GBM antibody

Aetiology

Ñ    Unknown

Ñ    Strong association between cigarette smoking and lung haemorrhage

Pathogenesis

Ñ    Glomerulonephritis ranging from focal segmental necrotising GN to crescentic GN

Clinical Features

Age

Ñ    May occur at any age

Ñ    More common in 3^rd decade and 6^th / 7^th decade.

Sex

Ñ    Males > Females

E&M

Ñ    Fever

Ñ    Weight loss

RS

Ñ    Haemoptysis

Ñ    Dyspnoea

KUS

Ñ    Oliguria

Ñ    Oedema

Ñ    Loin pain

Ñ    Haematuria

Examination

IS

Ñ    Cyanosis

RS

Ñ    Tachypnoea

Ñ    Inspiratory crepitations (crackles) throughout lung fields

KUS

Ñ    Evidence of renal failure

Investigations

Fluids

Urine

Ñ    Proteinuria

Ñ    Haematuria

Ñ    Red cell casts

Ñ    Granular casts

Blood

Immunology

Ñ    Anti glomerular basement membrane antibodies (anti GBM)

Imaging

Chest X-ray

Ñ    Alveolar shadowing:

Central and lower lung fields

Physiological studies

Ñ    Increased corrected carbon monoxide uptake K_CO:

Free haemoglobin in the alveoli leads to an increased value

Endoscopy

Bronchoscopy

Ñ    May show haemorrhage

Tissue Diagnosis

Transbronchial biopsy

Ñ    Intra-alveolar haemorrhage with haemosiderin laden macrophages and alveolar cell hyperplasia

Renal biopsy

Ñ    Early changes are focal and segmental proliferative glomerulonephritis

Ñ    Later the changes become diffuse with focal necrosis and extensive crescent formation

Management

Control

Drugs

Ñ    Steroids

Ñ    Cyclophosphamide

Biological Agents

Ñ    Plasma Exchange:

Removes circulating antibodies

Support

Equipment

Ñ    Dialysis

Biological Agents

Ñ    Transplantation may be successful if the patient does not have circulating antibodies

 

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