Goodpasture Syndrome - A Life-Threatening Autoimmune Disorder
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Goodpasture Syndrome - A Life-Threatening Autoimmune Disorder

Published on Jul 05, 2022 and last reviewed on Jul 06, 2023   -  5 min read


Goodpasture syndrome is a rare autoimmune disorder that affects the basement membrane of the lungs and kidneys. To know more about it, read the following article.

Goodpasture Syndrome - A Life-Threatening Autoimmune Disorder


In 1919, Ernest Goodpasture described this disorder for the first time, and it is named after him. Goodpasture syndrome is an acute group of disorders characterized by autoantibodies directed against the basement membranes of the lungs and kidneys. It is believed to be of genetic origin, but the combination of other factors also plays a role in the pathogenesis of this condition. The symptoms aggravate as the disease progresses. Earlier diagnosis of the condition helps prevent further complications.

What Is Goodpasture Syndrome?

Goodpasture syndrome is a pulmonary-renal syndrome where the person’s own antibodies are directed against their lung and kidneys. This happens because the body's immune cells mistakenly start attacking the collagen that is present in the basement membrane of the kidneys and lungs. This eventually causes severe inflammation and destruction of the pulmonary and renal cells leading to failure of those organs. It is a serious fatal complication and needs to be addressed earlier.

What Is the Etiology of Goodpasture Syndrome?

It is an autoimmune disorder, and it is proposed to be due to environmental insults in persons with a genetic predisposition. Few studies report HLA-DR15 as a triggering stimulus in the production of autoantibodies against the glomerular basement membrane.

The environmental factors which act as an initial insult include,

  • Smoking.

  • Drugs such as Alemtuzumab.

  • Cocaine inhalation.

  • Viral infections.

  • Exposure to chemicals such as organic solvents, hydrocarbons, etc.

What Is the Epidemiology of Goodpasture Syndrome?

It is more common in Whites than in the Black population, seen in younger men and older women. It shows bimodal age distribution, which is 20 to 30 years and 60 to 70 years, respectively, and accounts for one to five percent of all kinds of glomerulonephritis.

What Is the Pathogenesis of Goodpasture Syndrome?

Autoantibodies directed against the basement membrane will form antigen-antibody complexes, which, in turn, activate the complement system leading to tissue injury and inflammation. These bound autoantibodies are seen as linear deposition along the basement membrane, which can be visualized through immunofluorescence.

Why Do Pulmonary Symptoms Go Unnoticed?

In an otherwise healthy individual, the endothelium of the basement membrane acts as a barrier to these autoantibodies, preventing their binding with the alveolar basement membrane, which causes pulmonary symptoms. But in some cases, increased environmental insults such as smoking, upper respiratory tract infection, and bacteremia (a term that describes the presence of bacteria in the blood) can as well alter the barrier membrane leading to increased permeability, thus allowing the autoantibodies to enter and bind with the basement membrane leading to pulmonary symptoms.

What Are the Signs and Symptoms of Goodpasture Syndrome?

Initial symptoms include,

  • Fatigue.

  • Nausea.

  • Fever.

  • Breathing difficulty.

  • Coughing up blood.

As the disease progresses, the following symptoms can occur,

  • Anemia - Decreased red blood cell (RBC) count.

  • Hematuria - Blood in the urine.

  • Proteinuria - Protein in urine.

  • Oliguria - Decreased urine output.

  • Hypertension - Increased blood pressure.

  • Cyanosis - Bluish discoloration of the skin and mucous membrane.

  • Edema - Collection of fluid in the interstitial space.

How Is Goodpasture Syndrome Diagnosed?

A proper history of its symptoms and a heedful physical examination are imperative in the diagnosis of this disorder. The laboratory investigations include,

  • Urine Analysis - To detect the presence of protein in the urine (proteinuria), presence of blood in the urine (hematuria), and red blood cell (RBC) casts in the urine.

  • Blood Investigations - To check for antibodies (anti-glomerular antibodies) in the blood and anemia as a result of intrapulmonary hemorrhages.

  • Renal Function Test - To detect renal malfunction.

  • Chest X-Ray / Computerized Tomography (CT) Scan - To detect any pulmonary changes.

  • Renal Biopsy - To check for histopathological signs of glomerulonephritis and to detect the antibodies against the basement membrane.

What Are the Histopathological Findings of Goodpasture Syndrome?

Microscopic examination of the renal biopsy will reveal the presence of glomerulonephritis. In the advanced stages, there will be fibrosis of the crescents followed by sclerosis. The interstitial stroma will show fibrosis and tubular atrophy. Immunofluorescence studies convey the linear deposition of IgG antibodies and complement along the basement membrane. The histopathological diagnosis will help in assessing the survival prognosis by identifying the number of crescents.

Lung biopsy is usually not indicated because of the complexity of the biopsy procedure as well as the difficult tissue fixation process. Microscopic examination of the lung tissue will reveal the presence of hemorrhage with hemosiderin-laden macrophages in the alveolar spaces.

How Is Goodpasture Syndrome Managed?

Treatment for this condition is based on certain factors such as the age of the patient, the severity of the disease, associated health conditions, etc. However, the general treatment protocol includes,

  • Immunosuppressive Drugs - Drugs like Cyclophosphamide are prescribed to suppress the immune system from attacking the body's own cells.

  • Corticosteroids - Drugs like Prednisone to reduce inflammation and stop bleeding from the lungs.

  • Plasmapheresis - It is a process by which the plasma is separated from the blood, cleared off anti-glomerular antibodies, and replaced with healthy plasma. It is done daily until the anti-glomerular antibodies become undetectable in the blood.

In patients with severe presentation such as both glomerulonephritis and pulmonary hemorrhage, immediate hemodialysis and intubation for respiratory failure are suggested.

What Are All the Differential Diagnosis of Goodpasture Syndrome?

All the conditions that affect the kidney and lungs should be considered a differential

diagnosis. It includes,

  • Systemic Lupus Erythematosus (SLE) - It is an autoimmune disease that affects the skin, lungs, heart, kidneys, etc.

  • Microscopic Polyangiitis - This is a rare type of vasculitis that causes inflammation of the blood vessels.

  • Wegener’s Granulomatosis - Rare disorder which is characterized by inflammation of blood vessels in the nose, kidneys, lungs, etc.

  • IgA Nephropathy - This is a kidney disease in which IgA builds up in the kidneys leading to diminished kidney function.

What Is the Prognosis of Goodpasture Syndrome?

The prognosis of this condition has improved remarkably over the years because of the development of belligerent treatment therapies such as immunosuppressive drugs, corticosteroids, and plasmapheresis. The five-year survival rate is found to be 80 % after the introduction of these therapies. This array of diseases shows an overall good prognosis when it is diagnosed earlier and treated on time. The recurrence rate is very low, but possibilities of recurrence are reported.

What Are the Complications of Goodpasture Syndrome?

Complications such as decreased kidney function lead to kidney failure. 30 % of affected patients require long-term dialysis. In severe cases, renal transplantation is indicated.


Goodpasture syndrome is a rare autoimmune disorder that may rapidly result in perpetual damage to the lungs and kidneys. It may be fatal if not quickly diagnosed and treated. Addressing the condition earlier and seeking medical help at a prompt time will help in preventing further damage to the tissues. With the introduction of advanced treatment modalities, the prognosis of this spectrum of disorders has increased drastically.

Frequently Asked Questions


Is Goodpasture Syndrome Fatal?

Goodpasture syndrome is a life-threatening autoimmune disorder that can be fatal if not diagnosed and treated immediately. The syndrome can cause rapid damage to the kidneys and lungs. Hence, the condition has to be handled seriously with a proper treatment plan to prevent further damage.


Is Goodpasture Syndrome Inherited?

Genetic predisposition plays a significant role in the origin of Goodpasture syndrome. The ailment may be inherited and can run in families. Some other well-accepted proposals to cause Good pasture syndrome are smoking, drug abuse, viral infection, and long-time exposure to volatile chemicals.


How Can You Tell the Difference Between Wegener's and Goodpasture's?

Goodpasture and Wegener syndrome show the same clinical presentations in kidneys but differ in lung manifestations. Goodpasture syndrome causes bleeding in the lungs leading to pulmonary siderosis, while Wegener's syndrome causes granulomatous changes in the respiratory tract leading to cancer.


Is Goodpasture Syndrome a Vasculitis?

In Goodpasture syndrome, autoantibodies mainly affect the lungs and the kidneys. But studies have found evidence of vasculitis, the inflammation of the blood vessels in patients with Goodpasture syndrome. Biopsies have shown vasculitis in glomerular and pulmonary capillaries.


How Do You Get Goodpasture's Disease?

The exact etiology of Goodpasture disease is unknown. But the widely accepted fact is the combination of environmental and genetic factors. Insult to the basement membrane cells in the lungs and kidneys, smoking, drugs, viral infection, or chemical exposure will predispose the genetic events leading to Goodpasture syndrome.


Does Goodpasture Syndrome Go Away?

The symptoms of Good pasture syndrome can be controlled, and the disease progression can be halted with the use of immunosuppressive drugs and steroids. Sometimes the body may stop producing autoantibodies, but the damage in the lungs and the kidneys must be treated.


Which Autoantibodies Are Strong With Goodpasture's Syndrome?

The autoantibodies commonly associated with Goodpasture syndrome are anti-GBM (glomerular basement membrane) antibodies. These autoantibodies target the collagen in the kidney and lung basement membranes. Hence, Goodpasture syndrome is also known as anti-GBM disease.


Is Goodpasture Syndrome Autoimmune?

Goodpasture syndrome is an autoimmune disorder that rarely occurs, resulting from changes in the environment and genetic factors. The body's natural defending immune system produces autoantibodies against the basement membrane in the lungs and kidneys. This process will cause bleeding in the lungs and inflammation in the kidneys.


Can You Recover From Goodpasture Syndrome?

The five-year survival rate of Goodpasture syndrome is 80 % with appropriate treatment and follow-up strategy. Only a few patients will have long-term complications like complete kidney dysfunction requiring dialysis. Surgical kidney replacement is also an option to recover from Goodpasture syndrome.


What Type of Hypersensitivity Is Goodpasture Syndrome?

IgG mediates the typical hypersensitivity reaction in Goodpasture syndrome. As the autoantibodies are pointed towards the collagen IV, this reaction falls under type-II hypersensitivity based on Coombs and Gell's classification of antigen-antibody reactions. The complement system is activated to cause tissue damage.


How Much Is Goodpasture Syndrome Life Expectancy?

Previously, Goodpasture syndrome was considered fatal, with a 90 % of a death rate. However, about 50 % of patients have a long-survival rate with advancements in immune-modulating drugs and techniques like plasmapheresis.


Can Goodpasture Syndrome Come Back?

The recurrence of Goodpasture syndrome is infrequent but has been reported in a few people. The autoantibodies are recommenced against the lung and kidney tissues. The severity of the symptoms is less than the initial presentation, and treatment planning follows the same path.

Last reviewed at:
06 Jul 2023  -  5 min read




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