Goodpasture’s Syndrome, more accurately referred to today as anti-GBM disease, is a rare autoimmune small vessel vasculitis. In this condition, the immune system produces circulating antibodies that mistakenly attack a specific antigen found in the glomerular basement membrane (GBM) of the kidneys and the alveolar basement membrane (ABM) of the lungs.¹ In the kidneys, this immune attack causes rapidly progressive glomerulonephritis, leading to the leakage of blood (hematuria) and protein (proteinuria) in the urine, and may eventually result in renal failure. In the lungs, the same immune mechanism triggers alveolar hemorrhage, which can present as coughing up blood and shortness of breath.

Although “Goodpasture’s Syndrome” and “Goodpasture’s Disease” are older terms, they often refer to this anti-GBM antibody-mediated disease, especially when both glomerulonephritis and pulmonary hemorrhage occur together. If left untreated, the condition can be life-threatening.²

What does Autoimmunity mean?

It means the body turning against itself. In our body, some B-lymphocytes (a type of defending cells) convert into plasma cells and make specific proteins (antibodies) to fight against invaders. The immune system uses HLA or human leucocyte antigen as a tag to distinguish the body’s own cells from foreigners. However, in some instances, this mechanism gets disrupted. So, antibodies falsely perceive the body’s own cells as enemies, thus killing them and damaging the body.

How common is Goodpasture’s Syndrome?

Fortunately, it is a rare condition that affects one out of a million people.³ Though it can affect any age group, according to the National Kidney Foundation (NKF), white men aged 20 to 30 years are at higher risk of developing it.⁴

Cause of Goodpasture’s Syndrome

Goodpasture’s syndrome is caused by autoantibodies that target the α3 chain of type IV collagen—a protein essential to the structure of many tissues, including the glomerular and alveolar basement membranes.⁵ Along with the basement membrane, it is present throughout the body, particularly in skin, muscle, bone, blood vessels, and connective tissues.

According to the research, due to the preferential use of the same components, the alveolar membrane (lining of the lung) is quite similar to the glomerular membrane (lining of the kidney).⁶ This is why antibodies attack both of them simultaneously. As a result, inflammation occurs, and membranes get damaged. Consequently, the normal filtration process is impaired, leading to the leakage of water, blood cells, proteins, and inflammatory debris into the surrounding tissues

Who is at Risk?

The researchers have not fully figured out the exact reason behind the disease. However, they suggest that there are some risk factors for developing it, such as:

Exposure to vapors of certain chemicals such as hydrocarbons including gasoline.⁷
Metallic dust exposure in factory workers.
Lymphocyte-depletion therapy.
Tobacco smoking.
The disease tends to run in families, which is why researchers believe it has some genetic component.
Use of cocaine.⁸
After a viral infection such as the flu and sore throat.

Symptoms of Goodpasture’s Syndrome

The disease starts as vague symptoms such as fatigue and body aches. Along with that, nausea, vomiting, and loss of appetite may also occur. Since it is a rapidly progressing disease, in a matter of days, a lot of damage happens to the kidneys and lungs. Moreover, widespread damage to organs can cause loss of blood and the appearance of pallor.

Alveolar symptoms:

Damage to the lungs manifests as a spectrum of different symptoms, such as:

Cough & Dyspnea

The patient presents with a cough with or without blood, along with shortness of breath.

Chest Pain

Though uncommon, it may occur in some cases.

Respiratory Failure

In cases of extensive damage, bleeding in the lungs occurs, which leads to bluish discoloration of the body. The patient feels unable to breathe, and urgent assisted ventilation is required at this stage.

Glomerular Symptoms:

Damage to the basement membrane of the kidney results in the impairment of the filtration process. This leads to the appearance of the following symptoms:

Hematuria & Proteinuria

Leaking of protein and red blood cells causes red and foamy urine.

Flank pain

Due to the inflammation, the patient may experience pain in the back under the ribs.

Infection

Many important proteins start leaking out of the body including the one needed for making antibodies. Hence, the defense mechanism is impaired, leading to an increased risk of infection.

High Blood Pressure

As the kidneys are unable to filter out waste material, blood pressure rises, further damaging the kidneys.

Swelling of the Body

The presence of protein in the body keeps the fluid inside the vessels. Due to the decrease in proteins, fluids leak into tissue spaces, causing swelling in the body.

Glomerular damage in Goodpasture's Syndrome. — Illustration of Goodpasture’s Syndrome: Autoantibodies target the basement membranes in the kidneys, leading to inflammation and tissue damage.

Diagnosis of Goodpasture’s Syndrome

If doctors suspect the symptoms of Goodpasture’s syndrome, they will start by examining the patient. Important signs to look for include:

Increased rate of respiration
A rattling sound in the lungs (rales)
Appearance of rash
Enlargement of liver and spleen
Bluish discoloration on the body (Cyanosis)

After that, they may advise some tests such as:

Urinalysis:

It is the study of urine to check its characteristics. The presence of protein and blood suggests damage to the basement membrane.

Urine Albumin-to-Creatinine Ratio:

This ratio helps assess kidney function and detect kidney damage.

Blood Tests:

Blood samples are analyzed to check for anti-glomerular basement membrane (anti-GBM) antibodies, which are a hallmark of Goodpasture’s syndrome. Additionally, the glomerular filtration rate (GFR) may be calculated to evaluate kidney function.⁹

Imaging Test:

Chest X-rays show white patches on both lungs because of the damaged areas. The radiographic findings resolve within 2-3 weeks.

Bronchoscopy:

In bronchoscopy, the doctors utilize a flexible tube with a camera (bronchoscope) to visualize the lungs. By using this, they can look at the areas of hemorrhage directly.

Biopsy:

It gives the definitive diagnosis of the disease. The physician takes a sample from the lung or kidney and sends it to the lab. There, the histopathologist runs different tests on it and examines it under the microscope, hence confirming the diagnosis.

Blood sample for the diagnosis of Goodpasture's syndrome — Anti-GBM Antibodies in the blood are diagnostic for Goodpasture syndrome

The Triad of Good Pasture Syndrome

The Triad, in medical terms, means a group of three symptoms that occur together in a specific condition. Here, the triad consists of:¹⁰

The presence of anti-glomerular basement membrane antibodies
Pulmonary hemorrhage or bleeding in the lung
Glomerulonephritis or inflammation of the kidneys

Treatment of Goodpasture’s Syndrome

It is a serious condition that requires immediate aggressive medical treatment. The patient requires hospitalization with strict monitoring, and in some cases, they may be shifted to the intensive care unit or ICU. The goal of the treatment includes:

Fighting the Antibodies:

To combat the harmful antibodies that target the basement membrane, doctors administer immunosuppressive drugs. Cyclophosphamide remains the primary treatment choice for this purpose. Additionally, corticosteroids such as prednisolone are often administered intravenously to reduce inflammation and prevent complications like pulmonary hemorrhage (bleeding in the lungs). However, immunosuppression can increase the risk of infections. Treatment typically lasts for 6 to 12 months, but in cases where the condition is refractory or unresponsive, treatment may be extended, and alternative drugs like rituximab may be prescribed.¹¹

Control Hypertension & Fluid Buildup:

To decrease blood pressure, the patient needs drugs such as AEC inhibitors (angiotensin-converting enzyme inhibitors) or ARBs (angiotensin receptor blockers). These drugs not only help lower blood pressure but also protect the kidneys from further damage, supporting their ability to filter waste products such as urea and creatinine. This, in turn, helps reduce edema (swelling).

Plasmapheresis:

This is a method to clean the blood of destructive antibodies. It includes taking blood from a vein using a cannula and separating blood cells and plasma. After that, they replace the damaged plasma with healthy donor plasma. In some cases, the patient needs plasmapheresis daily for several weeks.¹²

Dialysis & Kidney Transplant:

If substantial damage to the basement membrane occurs, the kidneys stop working, hence, the patient may require dialysis or a kidney transplant to manage the glomerular disease.

Differntial Diagnosis of Goodpasture’s Syndrome

Some conditions that affect the lung and kidney (pulmonary-renal syndromes) are important in making differential diagnoses. These include:

Granulomatosis with polyangiitis (Wegener’s granulomatosis)
Systemic lupus erythematosus
Microscopic polyangiitis
Alport syndrome

The presentation of Wegener’s Granulomatosis is similar to Goodpasture’s syndrome. Hence, it is difficult to distinguish between them. Moreover, some cases of Goodpasture’s syndrome are also positive for anti-neutrophilic cytoplasmic antibodies (ANCAs), just like Wegner’s granulomatosis, thus confusing the diagnosis.

Prognosis of Goodpasture’s Syndrome

Early diagnosis and management lead to a better outcome. According to research, with proper management, the 5 years survival rate is 80%.
The course of treatment may take several weeks to up to 2 years. In case of severe lung injury, the patient may need to be put on a ventilator. The primary cause of death is pulmonary hemorrhage. On the other hand, the most significant long-term adverse outcome is renal failure.
Fortunately, once resolved, recurrence is very rare. In addition, Lifestyle modification such as quitting smoking and changing jobs helps to prevent recurrence.

Diffuse pulmonary hemorrhage — (Diffuse bilateral pulmonary hemorrhage in Goodpasture’s syndrome) **Image Courtesy:** Dr. Yale Rosen, taken from **Flickr** (**CC BY-SA 2.0**)

Goodpasture’s vs. Alport Syndrome

The presentation of the disease can mimic Alport Syndrome, another condition that harms the collagen. Key differences include:

Goodpasture’s syndrome	Alport syndrome
Anti-bodies destroy the basement membrane of organs.	The genetic mutation leads to impaired collagen formation.
It affects the kidneys and lungs.	It mainly affects the kidneys, eyes and ears.
Lungs are damaged in it.	Minimal to no lung injury.
It is an auto-immune condition.	It is a genetic abnormality.
Plasmapheresis and steroids for treatment.	Supportive treatment only.

Conclusion

Goodpasture’s syndrome is a condition in which our immune system turns against the body. It is a potentially life-threatening condition with extensive injury to the lungs and kidneys. If a patient presents with blood in urine or edema with difficulty breathing and coughing, suspect it. Suppression of the immune system and changing the plasma help reduce the damage. Severe cases may need dialysis and ventilatory support.

Refrences

1
Donaghy M, Rees AJ. Sequential studies of pulmonary function in Goodpasture’s syndrome. Q J Med. 1983;52(208):515-528.
2
McAdoo SP, Pusey CD. Anti-GBM (Goodpasture) disease: Pathogenesis, clinical manifestations, and diagnosis. UpToDate. Waltham, MA: UpToDate Inc. https://www.uptodate.com/contents/anti-gbm-goodpasture-disease-pathogenesis-clinical-manifestations-and-diagnosis. Accessed April 6, 2025.
3
McAdoo SP, Pusey CD. Anti-Glomerular Basement Membrane Disease. Clin J Am Soc Nephrol. 2017 Jul 7;12(7):1162-1172. doi: 10.2215/CJN.01380217. Epub 2017 May 17. PMID: 28515156; PMCID: PMC5498345.
4
National Kidney Foundation. (n.d.). Goodpasture syndrome. https://www.kidney.org/kidney-topics/goodpasture-syndrome
5
Borza DB, Netzer KO, Leinonen A, et al. The Goodpasture autoantigen: identification of multiple cryptic epitopes on the NC1 domain of the α3(IV) collagen chain. J Biol Chem. 2000;275(8):6030-6037.
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Jandl K, Mutgan AC, Eller K, Schaefer L, Kwapiszewska G. The basement membrane in the cross-roads between the lung and kidney. Matrix Biol. 2022;105:31-52.
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Bowman C, Ambrus K, Lockwood CM. Hydrocarbon exposure and anti-glomerular basement membrane disease. Kidney Int. 1984;25(5):689-693.
8
Rout P, DeVrieze BW. Goodpasture Syndrome. In: StatPearls. StatPearls Publishing; 2024.
9
Hellmark T, Segelmark M, Niles JL. Diagnosis and classification of Goodpasture’s disease (anti-GBM). J Autoimmun. 2014;48-49:108-112.
10
Ramaswami A, Kandaswamy T, Rajendran T, Aung H, Jacob CK, Zinna HS, Telesinge PU. Goodpasture’s syndrome with positive C-ANCA and normal renal function: A case report. J Med Case Rep. 2008;2:223.
11
Levy JB, Turner AN, Rees AJ, Pusey CD. Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression. Ann Intern Med. 2001;134(11):1033-1042.
12
Simpson IJ, Doak PB, Williams LC, Blacklock HA, Hill RS, Teague CA, Herdson PB, Wilson CB. Plasma exchange in Goodpasture’s syndrome. Am J Nephrol. 1982;2(6):301-311.

Goodpasture’s Syndrome: Autoimmunity Gone Wrong

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