Goodpasture Syndrome

Goodpasture syndrome is a rare but potentially deadly disorder that affects about 0.5 to 1.8 people per 1 million every year (Rout & DeVrieze 2024).

Recognising the symptoms of Goodpasture syndrome and escalating care immediately is crucial in preventing fatality (Rout & DeVrieze 2024).

What is Goodpasture Syndrome?

Goodpasture syndrome, which may also be referred to as anti-glomerular basement membrane (anti-GBM) disease, is a potentially life-threatening autoimmune disorder where the body produces antibodies that attack the lining of the lungs and kidneys (Rout & DeVrieze 2024; NKF 2024).

The disorder is characterised by:

• The presence of anti-GBM antibodies, with
  • Pulmonary alveolar haemorrhage (bleeding in the lungs), and/or
  • Glomerulonephritis (kidney inflammation).

(GARD 2025; NKF 2024; Lee 2023)

Without prompt treatment, symptoms can progress rapidly, leading to life-threatening bleeding in the lungs, renal failure and potentially death (Story 2018; NKF 2024).

What Causes Goodpasture Syndrome?

It’s unknown exactly what causes anti-GBM antibodies to develop, but they’re thought to be triggered by an environmental insult in a person with a pre-existing genetic susceptibility (GARD 2025).

Once anti-GBM antibodies are produced, they attack the collagen present in the alveoli and capillaries in the lungs, and/or the glomerulus (filtering units in the kidneys), causing bleeding in the lungs and inflammation in the kidneys (GARD 2025).

Potential environmental triggers include:

• Certain medicines (e.g. alemtuzumab)
• Cocaine inhalation
• Certain infections (e.g. influenza A2)
• Smoking
• Exposure to metal dust, organic solvents or hydrocarbons
• Extracorporeal shock wave lithotripsy (which is used to treat kidney stones).

(Rout & DeVrieze 2024; NKF 2025)

Those who have a genetic predisposition to the creation of anti-GBM antibodies appear to have specific human leukocyte antigen (HLA) subtypes (Rout & DeVrieze 2024).

Symptoms of Goodpasture Syndrome

Symptoms may include:

• Haemoptysis (coughing up blood)
• Cough
• Respiratory impairment
• Anaemia
• Joint pain
• Arthritis
• Chest pain
• Glomerulopathy
• Muscle pain
• Retention of baby teeth
• Purpura
• Renal insufficiency
• Retinal detachment
• Drug-induced vasculitis
• Oedema
• Hypertension
• Enlarged liver and spleen
• Cyanosis.

(GARD 2025; Rout & DeVrieze 2024)

Diagnosing Goodpasture Syndrome

A diagnosis is made upon the presentation of:

• Bleeding in the lungs, which can be detected via chest X-ray and lung biopsy, or bronchoscopy with fluid wash out
• Urinary symptoms (e.g. blood and/or protein in the urine), which can be detected via blood and urine tests
• Circulating anti-GBM antibodies, which can be detected via kidney biopsy.

(NKF 2024; Lee 2023)

There are several differential diagnoses that may need to be ruled out, including:

• Granulomatosis with polyangiitis
• Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
• Pneumocystis jiroveci pneumonia
• Respiratory failure
• Rheumatoid arthritis
• Systemic lupus erythematosus
• Microscopic polyangiitis
• Rheumatoid arthritis
• IgA-mediated disorders
• Acute glomerulonephritis
• Community-acquired pneumonia
• Cryoglobulinaemia
• Undifferentiated connective tissue disease
• Endocarditis
• Drug-induced vasculitis
• Alport syndrome.

(Rout & DeVrieze 2024; Kathuria 2023)

Treatment For Goodpasture Syndrome

Patients will often present critically ill and will require immediate haemodialysis, along with intubation in the case of respiratory failure. At this stage, a kidney biopsy may also be taken to confirm the diagnosis (Rout & DeVrieze 2024).

The primary goals of treatment are to:

• Quickly remove anti-GBM antibodies via daily plasmapheresis - a procedure wherein blood cells are separated from the plasma (which contains the anti-GBM antibodies), and the plasma is replaced with another solution, or treated and returned back to the body
• Prevent the body from creating more anti-GBM antibodies by administering immunosuppressive medicines
• Remove any environmental irritants that may have triggered the production of anti-GBM antibodies, e.g. via smoking cessation, or changing jobs if the patient is occupationally exposed to hydrocarbon.

(Kathuria 2023; Rout & DeVrieze 2024; Heitz 2018)

Patients may also require supportive care such as oxygen administration, mechanical ventilation and/or blood transfusion (Lee 2023).

If renal failure occurs, the patient will require dialysis or a kidney transplant (NKF 2024).

Post-discharge, the patient will need to continue treatment. Plasmapheresis is generally performed daily until anti-GBM antibodies are undetectable, and immunosuppressive therapy will continue for a further three to six months after cessation of plasmapheresis. Renal function will also be monitored long-term (Rout & DeVrieze 2024).

Overall, the five-year survival rate of Goodpasture syndrome is over 80%, however, it’s crucial that treatment is commenced immediately, as delays may lead to fatality (Kathuria 2023; Rout & DeVrieze 2024).