Goodpasture Syndrome: Understanding the Symptoms, Causes, & Importance of Treatment

What is goodpasture syndrome?

Goodpasture syndrome is a rare autoimmune disease in which the body’s immune system erroneously attacks its own tissues. Normally, your body makes antibodies (proteins that signal the immune system to attack) to fight infections. Goodpasture syndrome is characterized by antibodies that target specific proteins found in the lungs and kidneys. The syndrome can be slow or progress rapidly, and without treatment, it can lead to life-threatening bleeding in the lungs (pulmonary hemorrhage) or kidney failure.

Symptoms may be general at first and include fatigue, weakness, nausea, a loss of appetite, and pale skin, and then progress to include lung symptoms such as a cough, shortness of breath, chest pain, or coughing up blood, as well as kidney symptoms such as painful urination, reduced urination, blood in the urine, high blood pressure, and swelling.

Treatment includes measures to reduce damage to the lungs and kidneys, replace kidney function, as well as further support.

You should seek immediate medical care at an urgent care clinic or ER as this syndrome is fatal unless quickly diagnosed and treated.

Symptoms of goodpasture syndrome

Symptoms often develop rapidly over several days or weeks but can develop more slowly over many months.

Main symptoms

Vague, uncomfortable symptoms are most common, especially early on in the syndrome. These include:

• Fatigue: Feeling like you have little energy and get tired easily
• Weakness: Generalized loss of strength
• Nausea: Feeling sick to the stomach
• Loss of appetite: This may lead to unplanned weight loss over time.
• Pale skin: Blood loss can lead to pale skin due to a low red blood cell count (anemia).

Lung (pulmonary) symptoms

These occur before or at the same time as kidney symptoms in more than two-thirds of cases. In a minority of cases, the syndrome affects only the lungs or only the kidneys. Pulmonary symptoms can also occur months before kidney symptoms.

• Frequent dry coughing
• Coughing blood (hemoptysis): This can range from a few flecks to excessive amounts of blood.
• Shortness of breath: Blood and fluid in the lungs can result in difficulty breathing.
• Chest pain

Causes of goodpasture syndrome

Autoimmune attack on lungs and kidneys occur when antibodies (anti-glomerular basement membrane antibodies, anti-GBM antibodies) circulate and tag membranes or vessels of the lungs and kidneys for damage by immune cells. Kidney filtering structures (glomeruli) may become damaged, sometimes so severely that the kidneys can no longer perform their vital functions (kidney failure). Lung tissue is also often damaged, however, this damage is usually temporary.

Triggers

Autoimmune diseases often result from a combination of environmental and genetic factors. Exposures that can precipitate an autoimmune attack include:

• Respiratory infections: Viral infections, such as the flu, sometimes precede Goodpasture syndrome and may over activate the immune system. Pneumonia may also rarely lead to the syndrome.
• Chemical exposures: This may include hydrocarbons like gasoline, dry-cleaning chemicals, weedkillers like Paraquat, and hair dyes.
• Smoking

Who is most often affected

Groups more commonly affected by Goodpasture syndrome include the following.

• Younger adults: 20 to 30 years old
• Older adults: Over age 60
• Men
• Caucasians
• Those with inflammatory diseases: Goodpasture syndrome sometimes co-occurs with other inflammatory diseases such as Lupus and Granulomatosis with Polyangiitis (GPA, also known as Wegener’s Granulomatosis).
• Genetics: Cases of Goodpasture syndrome have been found to run in families, suggesting genetics may play a role. However, specific genes that may predispose individuals to the syndrome are still under investigation.

Treatment options and prevention for goodpasture syndrome

Reducing further lung and kidney damage

The prognosis is good when the syndrome is caught early and lung and kidney failure are able to be prevented. This can be done by the following measures.

• Plasma exchange (plasmapheresis): Using a special filtering process, physicians can temporarily remove blood from your body, eliminate harmful substances (such as unwanted antibodies, toxins, and metabolic wastes) out of the blood, and return healthy blood components back to your body.
• Immunosuppressant medications: Medications can also be used to slow the immune system in order to reduce tissue damage. Commonly used medications include cyclophosphamide (Cytoxan), corticosteroids (such as prednisone), as well as antibiotics to prevent infections since immunosuppressant medications can make you more susceptible.

Replace kidney function

Even with treatment, kidney function can decline to the point of kidney failure. When this occurs, the kidney’s normal filtering and fluid regulation functions must be replaced with medical interventions. These include:

• Blood pressure medications: Medications such as ACE-inhibitors (like lisinopril) or angiotensin-receptor blockers may be used to control blood pressure.
• Dialysis: If kidney failure develops, a dialysis machine can replace the kidney filtering functions. This usually involves going to a center for a few hours every other day.
• Kidney transplant: Transplant is a long-term alternative to dialysis in people with kidney failure.

Further treatment

Many people need further treatment, such as the following.

• Supplemental oxygen or blood transfusions: These supportive treatments may be needed during the height of illness.
• Diet changes: Depending on the extent of kidney damage, you may be counseled to limit salt and fluid intake to reduce swelling.

Prevention

You can help prevent the development of Goodpasture syndrome by adhering to the following.

• Stop smoking: Although Goodpasture syndrome can occur in the absence of tobacco exposure, continuing to smoke after surviving an episode of the syndrome can increase the risk of experiencing additional episodes. It is also important to carefully avoid secondhand smoke.
• Avoid chemical exposure: Avoid breathing in gasoline, glue, or hair dye to decrease your body’s exposure to chemicals that can precipitate the autoimmune attack.

When to seek further consultation for goodpasture syndrome

If you are coughing blood

Coughing up blood, although alarming, is usually an early sign of the disease which may result in earlier detection and treatment. If you cough up blood, it is important to promptly report this to your physician for further evaluation and treatment.

If you are having increasing difficulty breathing

Symptoms can develop rapidly. If you experience gasping for breath or blue skin tint (cyanosis) it is critical to seek immediate medication attention.

If you are producing less urine or bloody urine

Decreasing urine output, swelling of the extremities (edema), and bloody urine can be signs of kidney disease and should be reported to your physician. In some cases, kidney failure can progress rapidly and lead to death if not quickly diagnosed and treated.