Goodpasture’s Syndrome

Goodpasture’s Syndrome is an autoimmune disease that affects both children and adults. Read more about the disease, including its symptoms, causes, diagnosis, and treatment options.

Goodpasture’s Syndrome Definition

It is a very rare autoimmune disorder that causes the antibodies to attack the kidneys and lungs that ultimately lead to kidney failure and lung hemorrhage. This may permanently damage the kidneys and lungs, which may even result in the death of an individual. The kidney damage sometimes leads to Glomerulonephritis.

Ernest Goodpasture, an American pathologist, was the first person to discover and report this disease in 1919.

Goodpasture’s Syndrome Synonyms

It is also denoted by other names like:

  • Goodpasture’s Disease
  • Anti-Glomerular Basement Membrane Antibody Disease
  • Rapidly Progressive Glomerulonephritis with Pulmonary Hemorrhage
  • Pulmonary Renal Syndrome
  • Glomerulonephritis – Pulmonary Hemorrhage

Goodpasture’s Syndrome Incidence

It is a very rare disorder having a prevalence rate that ranges from 1 in 1,000,000 to 1 in 2,000,000 in European populations. This condition rarely attacks non-European populations. Individuals aged between eighteen years and thirty years, as well those aged from fifty years to sixty-five years, are more at risk of developing the syndrome. However, there are some cases where people aged anywhere between 4 years and 80 years have been positively diagnosed with it. Men are 6 times more prone to the disease as compared to women.

Goodpasture’s Syndrome Causes

The exact cause of this disorder is still unknown. It is a kind of autoimmune disorder that occurs when healthy body tissues are mistakenly attacked and destroyed by the immune system. The body of individuals affected by this disease develops certain substances that attack the collagen protein in the small air-sacs filtering units (Glomeruli) located in the lungs and the kidney.

In a few cases, certain viral respiratory infections may trigger this disorder. Inhaling hydrocarbon solvents can also lead to Pulmonary Renal Syndrome. In these cases, the immune system misidentifies various tissues as foreign chemicals or viruses and attacks them. The faulty response of the immune system damages the air sacs of the lungs and the filtering units of the kidney, causing hemorrhage and inflammation.

Various researches are being carried out to determine whether Pulmonary Renal Syndrome is a hereditary disease and what can be its inheritance pattern.

Goodpasture’s Syndrome Pathophysiology

Researchers believe this condition to be a Type II hypersensitivity reaction towards the Goodpasture’s antigens located on the kidney’s basement membrane as well as the pulmonary alveolus of the Type IV collagen. The immune system erroneously recognizes these substances as foreign and harmful. As a result, it starts producing antibodies (IgG) that attack the pulmonary alveolus of the lungs and the basement membranes of the kidney. These antibodies are known as anti-glomerular basement membrane antibodies. The glomerular basement membrane helps the kidneys to filter extra fluids and waste materials from the blood. Antibodies that work against this membrane are called anti-glomerular basement membrane. These antibodies damage the basement membrane of the kidney, leading to the damage of the organ.

Goodpasture’s Syndrome Risk Factors

Individuals with a history of exposure to hydrocarbons such as carbon tetrachloride, trichloroethane, ethyl ether and xylene are highly susceptible to this syndrome. Smokers or even former smokers also have increased risk of developing this syndrome. Such individuals are more likely to suffer from lung complications associated with this disease due to prior damage to their lung sacs.

People having some viral respiratory infections (such as influenza) may also develop this autoimmune disease.

Is Goodpasture’s Syndrome Contagious?

It is not an infectious disorder. However, experts believe that the initial stage of the disease may be triggered by certain kinds of viral infection that are contagious. The viral infection may initiate the autoimmune reaction within the body of an individual.

Goodpasture’s Syndrome Signs and Symptoms

The symptoms of this condition generally occur slowly over several months or years. In certain cases, however, the symptoms develop quickly over just a few days. The general signs of the condition include:

  • Fatigue
  • Weakness
  • Loss of appetite
  • Depression
  • Nausea and vomiting
  • Weight loss
  • Pale skin

Some of the more advanced symptoms are:

Lung Damage

Less severe lung damage symptoms include minor breathlessness and dry cough. The patient may experience these mild symptoms for a long period of time before more serious signs develop. At severe stages, the lung damage may trigger extreme breathing difficulty that the patient needs intensive care. The symptoms often deteriorate very rapidly. The lung hemorrhage due to Goodpasture’s Syndrome may even lead to anemia. People with a history of exposure to toxic fumes, individuals having lung infections and smokers are more prone to lung hemorrhage. The pulmonary function of an individual can be hampered by Goodpasture’s Syndrome.

Kidney Damage

The damage to the glomeruli due to by the condition leads to severe kidney inflammation or Nephritis. This symptom is usually not noticed until the patient’s condition deteriorates rapidly and the functioning of the kidneys stops completely within a few days. The Nephritic Syndrome sometimes causes another nephritic condition called RPGN or Rapidly Progressive Glomerulonephritis. Bloody urine is another symptom of this syndrome. It also leads to a reduction in the amount of urine output. The disease prevents the kidney from excreting the harmful products of the body (like urea) with urine. As a result, these products remain in the blood. This state is called acute renal failure. Renal failure symptoms cannot be observed before around 80% of the kidney function is lost.

The symptoms of this disorder, due to severe kidney damage, include high blood pressure, breathlessness and edema. The involvement of kidney generally causes Nephritic Syndrome. In some rare cases, this immunological disorder leads to Nephrotic Syndrome (NS) which is characterized by Proteinuria (abnormally high amounts of protein in urine) and severe edema. It also involves Hematuria and elevation in the level of blood pressure.

Goodpasture’s Syndrome Prevention

It is not possible to prevent this condition entirely as its exact causes are not known. However, there are some precautionary measures that can reduce the chances of an individual developing the disease. Smoking cessation helps with this purpose. One should also avoid inhaling the fumes from toxic materials like siphon gasoline also reduces the risks of having this syndrome.

Goodpasture’s Syndrome Diagnosis

It is not easy to identify the signs of this condition at an early stage which makes it very difficult to diagnose it at its initial stage. By the time the symptoms become evident, the disease reaches a very advanced stage with more than half of kidney function being lost.

Physical examinations often detect fluid overload and high blood pressure. Sometimes, abnormal sounds are audible from the heart and lung with the help of a stethoscope.

Individuals having the disease show abnormal urinalysis results with evidences of protein and blood in the urine. These reports may also detect abnormality in the red blood cells.

Some other diagnostic exams for this syndrome include:

  • Anti-glomerular basement membrane test
  • Chest x-ray
  • Kidney biopsy
  • Lung biopsy
  • Arterial blood gas
  • BUN
  • Creatinine (serum)

Goodpasture’s Syndrome Differential Diagnosis

The differential diagnosis of the condition involves distinguishing its symptoms from those of other disorders like:

  • Churg-Strauss Syndrome
  • Glomerulonephritis, Acute
  • Essential Mixed Cryoglobulinemia
  • Pneumocystis Carinii Pneumonia
  • Henoch-Schönlein Purpura
  • Microscopic Polyarteritis
  • Pneumonia, Community-Acquired
  • Rheumatoid Arthritis
  • Respiratory Failure
  • Wegener Granulomatosis
  • Undifferentiated Connective-Tissue Disease

Goodpasture’s Syndrome Treatment

There are no ways to completely cure this condition. Removing the destructive antibodies from the patient’s blood is the main goal of the treatment. Plasmapheresis is one of the main treatment options that remove all the blood from one’s body and replaces it with protein and fluid or donated plasma. This type of treatment usually continues from 3 to 6 months. The kidney and lung inflammation generally reduce once the harmful antibodies are removed from the body.

Various corticosteroid medications like prednisone can be used for treating Goodpasture’s Syndrome as these drugs are able to suppress the functioning of the immune system.

It is very important to keep the blood pressure in check for delaying kidney damage. One may use the ARBs or angiotensin receptor blockers and ACE inhibitors or angiotensin-converting enzyme inhibitors for controlling high blood pressure.

Doctors may ask patients to limit the fluid and salt intake to keep the swelling in check. In some cases, a doctor may also recommend a diet containing low to moderate amounts of protein. Patients are closely studied for any sign of kidney failure. Dialysis may be required in case of severe kidney failure.

An affected person may require a kidney transplant if he or she loses kidney function permanently. However, the transplant cannot be performed before the levels of the damaging antibodies are reduced effectively.

Goodpasture’s Syndrome Prognosis

The outcome varies from one patient to another. Diagnosing the disorder at very early stage is very important for a positive prognosis. The outlook is negative if the treatment begins at a later stage when the kidneys have already suffered serious damage. The degree of lung damage ranges from modest to severe, affecting the functioning of the lungs.

Goodpasture’s Syndrome and Pregnancy

This condition rarely affects women during pregnancy. The immunological condition requires renal transplant or long-term haemodialysis, which makes it unlikely for a woman having Goodpasture’s Syndrome to become pregnant. Haemodialysis and immunosuppressive agents can cause various maternal and fetal complications if a woman becomes pregnant while receiving treatments for this disease.

Goodpasture’s Syndrome Life Expectancy

This disease has a mortality rate of over 90%. However, various modern treatment options are helping patients to live longer. The life expectancy of a patient, after he or she is diagnosed with the disorder, varies according to the severity of the symptoms and the degree of kidney and lung damage. Kidney transplant can significantly increase the life expectancy of a patient.

Goodpasture’s Syndrome Support Groups

There are numerous forums and support groups located throughout the world that provide information and spread awareness about this life threatening disorder. These support groups also help patients and their families to fight the condition properly by providing them with useful guidelines. The contact details of a few support groups are given below:

Genetic and Rare Diseases Information Center (GARD)

P.O. Box 8126

Gaithersburg, MD 20898-8126

Phone: (888) 205-2311

Fax: (301) 251-4911

Website: http://rarediseases.info.nih.gov/GARD/Default.aspx

National Kidney and Urologic Diseases Information Clearinghouse (NKUDIC)

Clearinghouse

3 Information Way

Bethesda, MD 20892-3580

Fax: 703-738-4929

Phone: 1-800-891-5390

Email: nkudic@info.niddk.nih.gov

Website: www.kidney.niddk.nih.gov

Goodpasture’s Syndrome is a rare and life-threatening condition that may cause death of patients if not treated properly as early as possible. However, one can live a long and happy life with proper treatment at a very early stage. Renal transplant can also help a patient live a relatively long and normal life.

Goodpasture’s Syndrome Images

Following are some pictures that exhibit the external appearance of patients affected by this condition.

 

Picture 1 – Goodpasture’s Syndrome

 

Picture 2 – Goodpasture’s Syndrome Image