Goodpasture syndrome

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at December 2, 2016
StartDiseasesGoodpasture syndrome

Goodpasture syndrome is a condition characterized by inflammation of the glomeruli in the kidneys and hemorrhaging in the lungs. The glomeruli are structures in the kidney that filter wastes from the blood. Anti-glomerular basement membrane (GBM) antibodies are also present with this condition; these are proteins produced by the body to attack cells in the glomeruli.

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Definition & Facts

Goodpasture syndrome is an autoimmune disease, which means that the body mistakes its own cells and tissues for foreign invaders and attacks them. In the case of Goodpasture syndrome, the body attacks the collagen in the kidneys and the lungs. Collagen is a type of connective tissue that supports the framework of the body.

The disease strikes males more often than females. Though it can affect anyone of any age, it usually affects young adults or adults older than 60. It is more common in Caucasians than people of other races. Goodpasture syndrome is an extremely rare disease, and only 600 cases have been reported since 1919. A bout of Goodpasture syndrome can last for several weeks to two years. 

Goodpasture syndrome was named after Ernest Goodpasture, who described a patient with kidney failure and pulmonary hemorrhage in 1919. The disease is quickly fatal unless it’s treated, but the five-year survival rate is about 80 percent.

Symptoms & Complaints

The symptoms of Goodpasture syndrome may begin slowly and be nonspecific, then worsen quickly. People who have the syndrome experience nausea, vomiting, pallor, weakness, fatigue, and loss of appetite.

Sometimes the lungs are stricken before the kidneys, which gives rise to symptoms such as shortness of breath. The patient may have an unproductive cough or a cough that brings up blood. Bleeding in the lungs can be life-threatening if it is extensive.

When the kidneys are affected, the symptoms include blood in the urine, foam in the urine, and a burning pain upon urination. They may also have high blood pressure (hypertension); swelling in the legs, hands and feet (edema); and pain in the flanks, which are on either side of the body beneath the ribs. The patient may suffer chills, joint aches, and joint pain.

Causes

Medical experts do not know what causes Goodpasture syndrome, but people who smoke or use hair dyes seem to be more at risk than the general population. People who have had viral lung infections also seem to be at a higher risk.

Other people who are at risk have been exposed to the fumes of hydrocarbon solvents, dust that contains metal particulates, and illicit drugs like cocaine. There may be a genetic component to Goodpasture syndrome, and those with a family history may be at an increased risk.

Diagnosis & Tests

A doctor will give the patient a physical examination that checks for abnormal lung sounds, high blood pressure, and bleeding. They may then order several tests to help diagnose Goodpasture syndrome.

These include a urinalysis to detect protein in the urine or blood in the urine. High levels of blood or protein in the urine may be signs of kidney damage. Blood tests can reveal anti-GBM antibodies, while a chest X-ray can reveal spots in the lungs that signify pulmonary bleeding.

Sometimes a doctor may perform a kidney biopsy. During this test, a needle, guided by a computed tomography (CT scan) or ultrasound, enters the kidney and takes a small tissue sample, which is then studied under a microscope. If Goodpasture syndrome is present, there will be changes in the glomeruli and evidence of anti-GBM antibodies. The patient’s lungs can also be biopsied.

Treatment & Therapy

The treatment for Goodpasture syndrome needs to be aggressive if it is to prevent permanent damage to the kidneys and the lungs caused by bleeding, antibodies, high blood pressure and the buildup of fluid.

The doctor will prescribe drugs that suppress the patient’s immune system and keep it from attacking the collagen in the kidneys and lungs. One of these drugs is cyclophosphamide, which is also a chemotherapy drug that fights cancer. It can be injected or taken orally.

Corticosteroids are used to suppress the immune system and control bleeding in the lungs. In the case of pulmonary bleeding, the corticosteroids may be delivered intravenously. Prednisone is a drug chosen for Goodpasture syndrome.

The patient undergoes plasmapheresis in some cases. In this therapy, the patient’s blood is cleansed of anti-GBM antibodies. About 300 milliliters, or 10 fluid ounces of blood are taken from the patient and put in a centrifuge, which separates the plasma from the rest of the blood.

The plasma is the part of the blood that contains the damaging antibodies. The other blood components are then mixed with a synthetic plasma and put back into the patient’s body. This treatment is given to the patient every day for a few weeks, though the course of immunosuppressive drugs can last for at least six months to as long as a year. 

While they are being treated for Goodpasture syndrome, some patients need to have supplemental oxygen to help them breathe. In cases where the lungs are badly compromised, the patient may need to be placed on a ventilator. Blood transfusions may be necessary for some patents.

The combination of plasmapheresis and immunosuppressive drug therapy is successful in most cases. The pulmonary hemorrhage stops, and the lungs are not permanently damaged. However, the damage to the kidneys may be permanent and severe enough to require dialysis or a kidney transplant, but this complication affects less than 30 percent of people with Goodpasture syndrome.

Prevention & Prophylaxis

Though medical experts are not certain exposure to these toxins cause Goodpasture syndrome, people should not smoke, use illegal drugs, and be exposed to metal dust and gasoline fumes. They should use hair dyes sparingly.

Doctors may also recommend that patients cut down on their sodium intake. This helps reduce the edema, or swelling and stabilizes the blood pressure.