Goodpasture Syndrome: Understanding the Symptoms, Causes, & Importance of Treatment
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This article will review the symptoms, causes, management, and prevention of Goodpasture syndrome. Symptoms include fatigue, weakness, nausea, a loss of appetite, pale skin, lung symptoms such as a cough, shortness of breath, or coughing up blood, and kidney symptoms such as painful urination, reduced urination, and swelling, among others.
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.
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
Kidney (renal) symptoms
Inflammation of the kidneys (glomerulonephritis) can progress to kidney failure, in which the kidneys are unable to excrete waste products from the blood through the urine.
- Pain during urination (dysuria): Inflammation of the kidneys can lead to burning or difficulty urinating.
- Blood in the urine (hematuria): Darkened and/or foamy urine occurs in 40 percent of people due to losing blood and protein through damaged kidneys.
- Low urine output (oliguria): More severe kidney damage can lead to a reduced volume of urination.
- High blood pressure (hypertension): This can result from kidney impairment.
- Swelling (edema): Puffiness of the skin in any area, especially the legs, can result from excess fluid retention.
If left untreated, the condition may progress to:
- Chronic kidney disease: Which may progress to end-stage renal disease (ESRD)
- Lung failure
- Pulmonary hemorrhage: Excessive bleeding in the lungs
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.
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.
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
- 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.
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.
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.
Questions your doctor may ask to determine goodpasture syndrome
- Have you lost your appetite recently?
- Have you been feeling more tired than usual, lethargic or fatigued despite sleeping a normal amount?
- Are you sick enough to consider going to the emergency room right now?
- Any fever today or during the last week?
- Do you have a cough?
Self-diagnose with our free Buoy Assistant if you answer yes on any of these questions.
Dr. Becker is a psychiatry resident at the Mount Sinai Hospital. He received his undergraduate degree in Urban & Regional Studies from Cornell University (2012) and completed his medical degree at the Perelman School of Medicine at the University of Pennsylvania (2018). Prior to medical school, he worked as a pre-medical teaching assistant at Weill Cornell Medicine-Qatar, where he received an Excellence in Teaching Award. His research has focused on global health (including explanatory models of mental illness in Botswana, epidemiology of head trauma, and psychosocial aspects of HIV), adolescent and young adult mental health, and quality improvement. He enjoys communicating health-related science through writing and teaching and joined Buoy Health as a writer in 2018. In his free time he enjoys running, hiking, and exploring new places.
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- Schwarz MI. Goodpasture Syndrome. Merck Manual Professional Version. Revised Dec. 2018. Merck Manuals Professional Version Link
- Goodpasture syndrome. Genetic and Rare Diseases Information Center. Updated March 28, 2017. GARD Link
- Goodpasture syndrome. American Kidney Fund. American Kidney Fund Link