What is Goodpasture Syndrome
The name often sounds confusing for a disease. But it is named so in the honor of Ernest Goodpasture, an American pathologist of Vanderbilt University.
He is credited with describing this disease for the first time in 1919. The different names for this disease are an anti-GBM disease.
Goodpasture syndrome(also written in medical terminology as GPS) showing the presence of an antibody called anti-glomerular basement membrane antibody(anti-GBM Ab).
This antibody attacks the basement membrane present in the lungs and kidneys of the patient. This causes bleeding of lungs and kidney failure.
Thus this is a classic example of autoimmune disease, but it's very rare to be found. The human body has a type IV collagen and the antibody often referred to as Goodpasture's antigen, probably attacks the alpha-3 subunit of this particular collagen.
This autoimmune disease(here GPS) causes quick degradation of the patient's lungs and kidneys. The damage can also be a permanent kind of damage and hence leading to death.
The generally prescribed medications in this disease are corticosteroids and cyclophosphamides. It is helpful in the suppression of the hyperactive immune system. Also, the antibodies are removed from the blood by the process of plasmapheresis.
This disease is further studied under the specialisation of Rheumatology.
What are its signs and symptoms?
The disease i.e., Goodpasture Syndrome which is caused by antibodies(here anti-GBM Ab), basically attacks the basement membranes of lungs and kidneys. Hence the patient experiences malfunction in his lungs and kidneys, and some of the general symptoms are as follows:
- the feeling of discomfort, uneasiness or malaise;
- experience loss in body weight;
- the feeling of tiredness or lack of energy in their day to day lives;
- the patients often suffer from high body temperature and feverish attacks are common;
- squeezing in body muscles leading to chills;
- there are frequent pains and aches in the joints of the body.
Also, a study has revealed the following statistics associated with this disease:
- the patients experiencing malfunctions in both lungs and kidneys account for around 60 to 80 percent of the sample space
- the patients suffering from kidney malfunctions account for around 20 to 40 percent of the lot
- around less than 10 percent of the people taken into account suffers from only lungs attack.
As it has been seen that the problems in the patient's kidneys are often followed by ailments in his lungs. The visible signs indulge:
- blood in the sputum while coughing;
- experience pain in their chests (although the number is limited to less than 50 percent of total cases);
- excessive coughing;
- causing shortening of the breath of the patients affects with this disease.
Whereas in case of malfunctions in patient's kidneys, these symptoms are displayed:
- presence of blood is observed while urination;
- there is an increase in the protein content of the patient's urine;
- often the pressure in the blood raises leading to high blood pressure conditions;
- also, the urea present in the excreta(i.e., urine) is increased;
- there is often seen sudden swelling of the patient's limbs or on his face too.
What can be the possible causes of Goodpasture Syndrome?
As Goodpasture Syndrome has been classified into the category of rare diseases, there has been a major breakthrough in the exact cause of this disease.
However, it is believed that the responsible antigens or antibodies(here anti-GBM Ab) come into contact with the alveoli( a cup-shaped cavity in the lungs where gas exchange takes place) and the glomeruli basement membranes.
This occurs mainly because of the insult being caused to the blood movement in the blood vessels while taking blood and pumping of blood from the lungs. The probable reasons behind this disruption or insult may include:
- continuous inhalation of tobacco smoke;
- addiction to cocaine inhalation;
- inhalation of certain pollutants like metal dust;
- when the body is exposed to certain chemicals mainly those organic solvents like chloroform and certain hydrocarbons;
- when the body is infested with ailments like influenza A;
- when the person is exposed to ecosystems having high oxygen presence;
- conditions when the immune system attacks our body(i.e., sepsis);
- conditions of bacterial presence in our blood(i.e., bacteremia);
- when our body is treated with substances having anti lymphocytic antibodies and monoclonal ones in particular.
What may be the potential causes of this disease?
But it's believed that the accountable antigens or antibodies(here anti-GBM Ab) comes into contact with the alveoli( a cup formed a cavity within the lungs wherever gas exchange takes place) and also the glomeruli basement membranes.
This happens principally as a result of the insult being caused to the blood movement within the blood vessels, whereas taking blood and pumping of blood from the lungs. The probable reasons behind this disruption or insult might include:
- continuous inhalation of tobacco smoke;
- addiction to hard drug inhalation;
- inhalation of sure pollutants like metal dust;
- once the body is exposed to sure chemicals principally those organic solvents like chloroform and sure hydrocarbons;
- once the body is overrun with ailments like contagious disease influenza A;
- once the person is exposed to ecosystems having high atomic number 8 presence;
- conditions once the system attacks our body(i.e., sepsis);
- conditions of microorganism presence in our blood(i.e., bacteremia);
- once our body is treated with substances having anti lymphocytic antibodies and organism ones in particular.
Pathophysiology: - Study of overall changes caused by Goodpasture syndrome :The antibodies chiefly responsible for the syndrome i.e., anti-GBM Ab, produces a number of abnormal plasma cells. Further as already discussed, these anti-GBM antibodies come into contact with the alveoli, a cavity within the lungs and also with the glomeruli basement membranes and attack them.
The antigenic determinants also called epitopes, the reactive ones are bound by those anti-GBM antibodies which in turn activates the attached cascades complementing them.
The activation causes the death of tagged cells. The T cells, which are developed in the thymus gland and are responsible for immune response, also an equal partner in this crime. Generally, the reaction happening is an example of a type II hypersensitivity reaction.
How Goodpasture Syndrome could be diagnosed?
Often the rare diseases are difficult to be detected and diagnosed. The same is in the case of Goodpasture Syndrome as the conditions are rare. The reason being it could be the aftereffect of various other diseases.
Generally, a biopsy provides a conclusive report of affected tissues. A kidney biopsy gives an accurate diagnosis of the presence of the responsible antibodies in the sample of the organ under observation.
Around 33 percent of the patients show positive results for the presence of antineutrophilic cytoplasmic antibodies which is usually in their bloodstream, apart from the normal anti-GBM antibodies responsible for the Goodpasture Syndrome.
Those antibodies are generally present before the anti-GBM antibodies are detected ranging from few months to years even. If the disease is diagnosed at an earlier stage, the treatment is feasible else at the later stages the resultant on the patient is grave and deadly.
What could be the possible treatments for GPS?
The best medication preferred for the treatment of Goodpasture Syndrome is plasmapheresis. This is referred to as the process of separation or bifurcation of the various components present in the blood of the infected person.
This segregation is made on the basis of varying weights of the different components present in the blood after it is being passed through a centrifuge.
The antibodies i.e., anti-GBM Ab, responsible for attacks on basement membranes of the affected person's lungs and kidneys, are present in the plasma( a component of blood).
Thus during plasmapheresis, this component of blood is segregated and filtered out. The other components of blood which were separated from the likes of Red blood cells, White blood cells, and the platelets could be again used into our body back after being recycled and then would be reinfused intravenously.
Certain immunosuppressive drugs like cyclophosphamide, prednisone, and rituximab, are also prescribed by the medical practicing correspondents to the patients affected with the disease.
They are corticosteroid drugs which prevents the further growth of those harmful antibodies or the new anti-GBM antibodies and protect more damages to the basement membranes of lungs and kidneys.
Azathioprine may also be recommended by the doctors to control remission as these immunosuppressants are comparatively less toxic in nature.
Prognosis: - Predicting the expected development of Goodpasture Syndrome :If the disease is diagnosed in the earlier stages, and correct treatment procedures are followed, more than 80 percent of affected patients stay alive for at least five years.
Whereas very few individuals need to undergo dialysis for a longer time frame (the number accounts for around less than 30 percent of the lot).
There are chances of hemorrhages in the lungs or maybe advanced kidney failures leading to the death of an individual, in cases of no treatment of the individual infected with this disease.
A study was conducted in Australia and New Zealand gave a shocking observation. It showed that the patients who had undergone renal replacement therapy (including dialysis), their chances of survival increased by around six years.
Epidemiology: - patterns of health and disease conditions :
As the disease, Goodpasture Syndrome is a kind of rare disease, the following conclusions can be deduced about this disease:
- the worst affected people with this disease are the Maoris from New Zealand;
- the male counterpart is more likely to be affected with this disease than the females; as in the case of other autoimmune diseases;
- also, the white people are more susceptible to Goodpasture Syndrome in comparison to the blacks;
- the rate of people affected by this disease-account for approximately 0.5-1.8 people per million in the continents of Europe and Asia;
- a study has revealed the two age groups of people in which the disease largely develops in the body. They are 20-30 years and 60-70 years.