WHAT IS GOODPASTURE SYNDROME?
- Goodpasture syndrome (or anti-GBM disease) is a rare and life-threatening autoimmune disease that damages the lungs and kidneys. It occurs when the immune system mistakenly attacks a protein called collagen because it recognizes it as a foreign substance.
- Usually, in Goodpasture syndrome, it is seen that the body produces proteins (antibodies) that attach to collagen in parts of the lungs and kidneys. When they bind to collagen, these antibodies cause severe inflammation and destruction of these tissues.
- Anti-GBM disease can affect only the kidneys or both the lungs and kidneys (very rarely only the lungs are affected). The main reason why these organs are affected is that the basement membrane that is targeted or attacked in this disease is only accessible to the antibodies present in the kidneys and lungs and not of the other organs.
- Anti-GBM disease that only affects the kidneys of a person is called anti-GBM glomerulonephritis. This is because there is inflammation, which occurs in the glomeruli (filters) of the kidneys.
- Anti-GBM disease that affects both the kidneys and the lungs is often referred to as Goodpasture syndrome or Goodpasture disease
What are the symptoms of Goodpasture syndrome?
Goodpasture syndrome is a pulmonary-renal syndrome. It could affect both the lungs and the kidneys. Symptoms of the disease include coughing up blood, shortness of breath, fatigue, and anemia. Lung symptoms usually appear first in these cases. The symptoms include:
- Shortness of breath, chest pain, cough and crackles when breathing.
- Fatigue, Fever, Nosebleeds and coughing up blood.
- Pale (pale) skin.
Symptoms of Goodpasture syndrome caused by kidney damage include:
- Anemia, Blood in the urine (hematuria).
- Decreased urine output (urinary incontinence), High Blood Pressure.
Nausea and vomiting. If kidney disease is very severe, patients may notice that they only urinate a small amount and may also experience nausea and vomiting. Treatment includes medication and a procedure called plasma transfer. This procedure removes plasma containing these harmful antibodies and replaces it with healthy plasma.
Is GBM curable?
In the case of GBM, plasmapheresis is usually done in 2-3 weeks. Depending on how your body responds, you may need to continue taking medicines that weaken your immune system for up to 9 months. After treatment, the anti-GBM disease rarely comes back.
How is Anti-GBM treated?
Standard treatment for GBM disease includes electrophoresis, for rapid removal of pathogenic autoantibodies, cyclophosphamide, and corticosteroids, to further inhibit autoantibody production and ameliorate target organ inflammation.
Does Goodpasture syndrome go away?
The red and white blood cells are generally mixed in a plasma substitute and returned to the body. Usually, plasmapheresis is continued daily for several weeks. Goodpasture syndrome can last several weeks or up to two years but it does go away.
Is anti-GBM disease hereditary?
As with many other autoimmune diseases, the anti-GBM disease is thought to be initiated in individuals with a genetic predisposition to certain types of environmental stimuli. There is some evidence that there is a genetic component of the anti-GBM disease.
What causes anti-GBM?
Anti-GBM disease is an autoimmune disease and this disease occurs when the immune system mistakenly attacks and destroys healthy tissues in the body. People with this syndrome develop substances that attack a protein called collagen in the small air sacs of the lungs and the filtering units (glomeruli) of the kidneys.
How common is Goodpasture syndrome?
Goodpasture syndrome was first described in 1919 and is very rare. It is estimated that there are fewer than two cases per million people. The syndrome is found to affect men more often than women. It usually begins between the ages of 20 and 30 or after 60.
Who is most likely to get glomerular basement membrane disease?
Glomerulonephritis due to anti-GBM antibody disease is rare. It happens to less than 1 in a million people. It usually affects young Caucasian males (ages 15 to 35). After young men, this disease occurs most often in the elderly (in the late 50s and older, more women than men). It is said to be very rare in children.
What Causes Goodpasture’s Syndrome?
- In Goodpasture’s syndrome, an autoimmune disease, antibodies produced by the immune system attack collagen. Collagen is a protein with many functions. It is part of the structure of many tissues and among other things helps blood clot.
- Goodpasture’s syndrome attacks the collagen in the kidney’s glomerular basement membrane (GBM). These anti-GBM antibodies also attack the collagen in the air sacs of the lungs, destroying the tissue and causing bleeding and difficulty breathing.
- The cause of Goodpasture syndrome is unknown. It can result from a combination of environmental factors and genetics. 8-90% of people with Goodpasture syndrome have a type of human leukocyte antigen (HLA) called HLA-DR15.
- HLA are proteins that help the immune system distinguish between the body’s own tissues and foreign (or invading) material. In people with HLA-DR15, the antigen does not function properly.
- Goodpasture may develop after an infection such as a cold or the flu. People who smoke cigarettes, use cocaine, or are exposed to metal dust or hydrocarbon chemicals (such as methane or propane) are more likely to develop Goodpasture syndrome. Scientists believe these environmental factors may cause the disorder in people with her HLA-DR15.
How do health care providers diagnose Goodpasture syndrome?
Your doctor will examine you and ask you about your symptoms. To diagnose Goodpasture Syndrome, providers will order:
Diagnosing GP is often difficult because many other diseases can cause symptoms of a wide variety of conditions, and the condition itself is rare. The most accurate means of diagnosis is a biopsy of the affected tissue, especially the kidney. This is because it is the most suitable organ to sample for the presence of anti-GBM antibodies. Other means are-
- Blood tests check how the kidneys are functioning and look for antibodies in the blood.
- A urine test to check for blood or protein.
- A CT scan or chest x-ray to look for lung damage. bronchoscopy to examine the lungs
- Additionally, serological testing for ELISA testing is usually performed by searching for the alpha 3-NC1 domain region of collagen IV to avoid false-positive results when there is significant suspicion of disease.
How are anti-GBMs treated?
- Once the diagnosis is confirmed, treatment includes several different parts. Anti-GBM disease can be life-threatening due to hemorrhage in the lungs and can rapidly progress to renal failure, so it is important to start treatment as soon as possible.
- If the lungs are affected, give oxygen as needed to keep oxygen levels high. A temporary breathing tube may be required. If the kidneys are severely damaged, they may not be able to function adequately to do what they need to do and dialysis may be required to help them do their job. , balancing electrolyte and acid-base levels in the blood, and removing toxins/waste products from the body.
- Plasmapheresis. This is a procedure that removes anti-GBM antibodies from the bloodstream. Also called plasmapheresis or PLEX. Human blood is passed through a machine that removes antibodies from the blood, and then the (antibody-free) blood is returned to the body. Treatment lasts several hours and usually, he is done every 1-2 days for about 2 weeks.
- Immunosuppressive therapy (drugs that suppress the immune system) is used to weaken or prevent the immune system
- Corticosteroids – Usually this is given as a high-dose infusion of a drug called methylprednisolone (salmeterol) daily for three days. Oral (oral) drug therapy, such as prednisone, is usually continued after the injection and tapers off over 3 to 9 months. This drug reduces inflammation caused by the immune system, which contributes to kidney and lung damage. Cyclophosphamide (Cytoxan) – This is an immunosuppressive drug that can be given as a monthly infusion or as a daily oral (oral) dose. This usually lasts at least 2-3 months and up to 6 months. This drug helps prevent the immune system from making more anti-GBM autoantibodies.
- Rituximab (Rituxan) – This is an immunosuppressive drug given as an intravenous infusion, usually 2-4 doses 2-4 weeks apart. Although this drug has not been evaluated in studies, it has been used in some patients when cyclophosphamide is not a suitable option or in addition to cyclophosphamide.
- Checking anti-GBM antibody levels in the blood after starting treatment is important to ensure that the antibodies are cleared from the blood and not returned. It also helps determine how long to continue treatment.
- It is important to avoid exposures that can cause or induce disease recurrence. Relapses and relapses are rare, but inhalation injury (exposure to chemicals such as organic solvents) and smoking can cause disease relapses. Avoiding smoke and other inhaled chemicals is very important.
Can Goodpasture Syndrome be prevented?
- Goodpasture syndrome may not be prevented. However, you can lower your risk by avoiding cigarettes and chemicals that can cause damage.
- Your healthcare provider may do blood tests to look for the HLA-DR15 antigen. If you know you have the HLA-DR15 antigen, have regular check-ups with your doctor to monitor your health. If you smoke, talk to your healthcare provider about how to quit smoking.
- Frequent exposure to gasoline, kerosene, tar, or asphalt increases your risk of developing this condition. If possible, these hydrocarbons should be avoided.
What is the outlook for people with Goodpasture Syndrome?
Goodpasture’s syndrome causes life-threatening pulmonary hemorrhage. Without treatment, this bleeding can be fatal. It can also cause kidney failure. If diagnosed early, treatment is effective. After treatment, see your doctor regularly to monitor your kidney function.
Some people with Goodpasture syndrome have long-term kidney problems. Dialysis may be required and eventually, a kidney transplant may be required.
What are the chances of getting better?
- Anti-GBM disease progresses very rapidly in most patients. One of the most important impacts on the health status of people with the anti-GBM disease is the promptness of treatment.
- Kidney disease or loss of kidney function causes many symptoms only when kidney function is severely impaired, so it is important to avoid serious injury or damage, which occurs in half to two-thirds of the world’s population. Many people are unaware that they have this disease.
- The disease can lead to kidney failure. Although the severity of renal damage at diagnosis is one of the best predictors of a patient’s status, some people with renal failure who initially require dialysis improve with treatment and have long-term survival.
- Some people do not need dialysis. However, people with good kidney function 6 to 12 months after onset are generally doing very well because the disease does not usually recur.
- Once someone has an anti-GBM disease, recurrence of the disease is rare. However, another type of autoantibody called ANCA (short for anti-neutrophil cytoplasmic antibody) is also found in the blood of 20-30% of patients with the anti-GBM disease.
- ANCA antibodies are associated with another disease, ANCA vasculitis. 20-30% of patients with the anti-GBM disease who also have ANCA antibodies in their blood are at a much higher risk of disease recurrence or future recurrence.
- For this reason, all patients with anti-GBM disease should have their ANCA tested. need to do it. Most people with the anti-GBM disease who do not have ANCA antibodies rarely have recurrences (about 2-3%) once they develop the disease.