Goodpasture ‘s syndrome is a rare but serious autoimmune disease that primarily affects the lungs and kidneys. The disease cannot be cured.
What is Goodpasture Syndrome?
Goodpasture syndrome was first described by American pathologist Ernest William Goodpasture in 1919. He drew the picture of a certain form of kidney inflammation combined with pulmonary hemorrhage.
Today it is clear that the kidney inflammation is a rapidly progressing glomerulonephritis. Goodpasture’s syndrome is a type II autoimmune disease in which antibodies are formed against components of blood vessels, particularly in the kidneys and alveoli. Type II autoimmune diseases belong to the type II allergies. These are allergies of the cytotoxic type.
According to abbreviationfinder.org, the body forms immune complexes against cell-bound antigens. As a result of the immune reaction that then takes place, the body’s own cells are destroyed. The disease is extremely rare. There is a maximum of one case per 1,000,000 people per year. The disease is most common between the ages of 20 and 40. Men are affected twice as often as women.
Causes
As already described, Goodpasture’s syndrome is a type II autoimmune disease. The body of the affected patient produces antibodies against the so-called Goodpasture antigen. In the case of the sick person, this is located in the alveoli of the lungs and in the basal membrane of the kidney. The basement membrane is a thin layer of tissue in the kidney corpuscles. The antibodies attack and destroy the Goodpasture antigens within these structures.
This process causes severe inflammation in the kidneys and lungs, which severely impairs the function of the organs. While the kidneys are always affected, lung involvement is not obligatory. Goodpasture’s syndrome is therefore also referred to as glomerulonephritis with lung involvement. Pre-existing lung disease, smoking, and previous exposure to hydrocarbons increase the risk that the disease will also damage the lungs.
Symptoms, Ailments & Signs
Those affected are symptom-free for a relatively long time. At the beginning, the disease only manifests itself through uncharacteristic symptoms such as loss of appetite or vomiting. Later, the picture of progressive glomerulonephritis is the focus of the symptoms. Damage to the small vessels in the kidney corpuscles causes proteins to pass into the urine.
Edema develops as a result of the loss of protein. These are mainly noticeable through swelling in the area of the eyes and later also in the area of the lower legs and ankles. Protein and possibly also blood are detectable in the urine.
If more red blood cells get into the urine through the damaged vessels, it appears pink to reddish in color. The loss of blood can lead to anemia with symptoms such as hair loss, tiredness and exhaustion. If left untreated, glomerulonephritis quickly leads to terminal renal insufficiency with acute renal failure.
Symptoms of renal failure include itching, bone pain, edema, headache, fatigue, heart failure, or gastrointestinal discomfort. Lung involvement causes shortness of breath and coughing. In the late stages, those affected cough up blood. Here, too, anemia can develop due to the blood loss or an existing anemia can be aggravated. In addition, the bleeding within the lungs leads to iron deposits and thus to pulmonary siderosis.
Diagnosis
If Goodpasture’s syndrome is suspected, antibodies are determined using indirect immunofluorescence. Indirect immunofluorescence is used to examine whether the patient’s blood serum contains antibodies against the body’s own cells. For this purpose, the patient’s blood serum is placed on a cell substrate and washed off again after a short time. Only bound antibodies remain on the cell substrate.
An antibody labeled with fluorochrome is then bound to the substrate. This also binds to human antibodies. If antibodies have now adhered to the substrate in the first step, the second antibodies that have just been added now bind to these antibodies. These antibody complexes can be detected with a fluorescence microscope.
X-rays of the lungs can show damage. A lung biopsy may be done. A kidney biopsy can also be used to diagnose Goodpasture’s syndrome. Crescents can then be detected in the kidney tissue. These are absolutely typical of the autoimmune disease.
Complications
The symptoms and complications of Goodpasture’s syndrome vary greatly and in most cases only become apparent in the final stages of the disease. This leads to vomiting, diarrhea and loss of appetite. The loss of appetite can also lead to malnutrition, which is a very threatening condition for the patient’s health.
The kidneys are also damaged, so that in the worst case it can lead to kidney failure. In this case, the patient then needs dialysis to survive. There are also complaints about the eyes, which are often swollen. The patient feels tired and sick and suffers from hair loss. Headaches and body aches are also common. Furthermore, the respiratory tract becomes infected, which can lead to breathing difficulties and shortness of breath.
It is not uncommon for coughing up blood to occur. The complaints put an extreme strain on the patient’s everyday life and lead to a reduced quality of life. Treatment of Goodpasture’s syndrome is imperative, otherwise death will occur. There are no other complications with treatment, and the mortality rate is about 20 percent. If the disease is defeated, no further ailments occur.
When should you go to the doctor?
Since Goodpasture’s syndrome does not heal itself, a doctor must be consulted in any case. This significantly increases the life expectancy of the patient, although the disease itself cannot be cured. A doctor should be consulted if persistent vomiting and loss of appetite occur. Swelling in the eye or protein in the urine can also indicate Goodpasture’s syndrome and should always be examined by a doctor.
Many patients also suffer from blood loss and thus permanent fatigue and exhaustion. Furthermore, a visit to the doctor is necessary if the person concerned often suffers from headaches or bone pain. It also causes itching and discomfort in the stomach and intestines.
If Goodpasture’s syndrome is not treated, breathing difficulties also occur, which must be examined in any case. The first examination of the syndrome is carried out by a general practitioner in most cases. In the further treatment, however, the use of different specialists is necessary to alleviate the symptoms.
Treatment & Therapy
If left untreated, Goodpasture’s syndrome is always fatal. Even with therapy, the mortality rate used to be up to 90 percent. Today, the prognosis has improved significantly due to the use of glucocorticoid therapies. Cortisone preparations and additional immunosuppressants are used. These inhibit the function of the immune system. Preparations such as azathioprine or cyclophophamide are used.
Plasmapheresis can have a supporting effect. In plasmapheresis, the patient’s blood plasma is completely exchanged using a plasmapheresis device. The antibodies against the Goodpasture antigen are thereby eliminated. Smoking should be stopped immediately if the lungs are involved. It is also advisable to treat any lung infection immediately with antibiotics.
Treatment for Good Pasture Syndrome takes eight to twelve months. With the therapy, the chances of survival of the affected patients have increased drastically. Mortality has been reduced to less than 20 percent. However, the disease cannot be cured. Recurrences, so-called rebounds, are possible at any time.
Outlook & Forecast
There is no complete cure for Goodpasture’s syndrome because it is a genetic disease that can only be treated symptomatically. Without treatment, the syndrome leads to premature death in most cases. Some of the symptoms can be alleviated with the help of immunosuppressive drugs, although those affected are dependent on lifelong therapy.
If the person concerned smokes, the symptoms usually worsen and life expectancy is reduced. Goodpasture’s syndrome also generally severely limits the patient’s life. The affected person cannot participate in sporting activities and should not expose themselves to any physical exertion. This can also delay development in children, leading to growth or developmental disorders.
It is not uncommon for Goodpasture’s syndrome to be associated with psychological complaints or even depression that occur as a result of the disease. Despite treatment, those affected often suffer from severe pain in everyday life and can therefore not easily pursue normal activities. Due to the lack of appetite, there are often deficiency symptoms that have to be compensated.
Prevention
Since it is not known what causes the formation of antibodies against the body’s own cells, Goodpasture’s syndrome cannot be prevented. Since the disease is fatal without treatment, early diagnosis is very important. This is the only way to prevent serious organ damage and those affected can lead a largely normal life.
Aftercare
In the case of Goodpasture’s syndrome, the options for aftercare are very limited. Since the disease cannot be cured, those affected usually depend on lifelong therapy to relieve the symptoms. Self-healing cannot occur in this case.
Furthermore, the life expectancy of the patient is usually significantly reduced and limited by Goodpasture’s syndrome. In most cases, those affected with Goodpasture’s syndrome are dependent on taking medication. It is important to ensure that it is taken regularly, whereby possible interactions with other medications must also be taken into account.
In case of doubt, a doctor should always be consulted. In the case of children, the parents are primarily responsible for the correct and, above all, regular intake of medication. Regular blood tests are also necessary in order to detect disorders of the immune system at an early stage.
In general, sufferers of Goodpasture’s syndrome should always protect themselves from diseases and infections in order not to unnecessarily burden the immune system. Infections must be treated immediately with antibiotics, and alcohol must not be consumed while taking antibiotics. Furthermore, contact with other people affected by Goodpasture’s syndrome can also be useful.
You can do that yourself
Goodpasture syndrome cannot be treated through self-help options. Those affected are dependent on medical treatment, although it cannot be ruled out that the patient may die because the disease leads to kidney failure in many cases. Even after successful therapy, the disease can come back.
Many sufferers need psychological support because of the disease. This can mainly be given by friends and family. In serious cases, a psychologist or therapist can also be consulted.
If a child is suffering from Goodpasture’s syndrome, a clarifying and detailed discussion about the disease must be held in order to inform the child about the possible course of the disease. Talking to other people affected can also help and psychological problems and depression can be avoided.
Since the patients suffer from severe limitations and pain in their everyday lives, the body should always be protected. Strenuous work should therefore be avoided in order not to aggravate the symptoms. Furthermore, the patient should eat and drink regularly despite loss of appetite in order to avoid deficiency symptoms.
