Granulomatosis with polyangiitis (GPA): symptoms, perspectives and more



What is this condition?


Granulomatosis with polyangiitis (GPA) is a rare disease that inflames and damages small blood vessels in many organs, such as the kidneys, lungs, and sinuses. Inflammation limits blood flow and prevents enough oxygen from reaching your organs and tissues. This affects how well they work.


Tissue inflammations, called granulomas, form around the blood vessels. Granulomas can damage organs.


GPA is one of several types of vasculitis, a disorder that causes inflammation in the blood vessels.


GPA was previously known as Wegener's granulomatosis.


What are the symptoms?


GPA sometimes causes no symptoms at the beginning of the disease. The nose, sinuses and lungs are usually the first affected areas.


The symptoms that develop depend on the organs involved:




  • Nose. Symptoms may include nosebleeds and scabs.


  • The paranasal sinuses Sinusitis or congestion or nasal discharge may develop.


  • Light. It may include cough, bloody phlegm, difficulty breathing or wheezing.


  • Ears. You can experience ear infections, pain and hearing loss.


  • The eyes Symptoms may include redness, pain or changes in vision.


  • Skin. Sores, bruises, or rashes may develop.


  • The kidneys You may have blood in your urine.


  • Joints You may experience swelling and pain in the joints.


  • Nerves. It may include numbness, tingling or shooting pains in the arms, legs, hands or feet.


More generally, symptoms throughout the body include:



  • fever

  • fatigue

  • malaise, called malaise

  • night sweats

  • aches and pains

  • weightloss


What causes this condition?


GPA is an autoimmune disease. This means that the body's immune system mistakenly attacks its own healthy tissues. In the case of GPA, the immune system attacks the blood vessels.


Doctors do not know what causes the autoimmune attack. Genes do not seem to be involved and GPA rarely occurs in families.


Infections may be involved in triggering the disease. When viruses or bacteria enter your body, your immune system responds by sending cells that cause inflammation. The immune response could damage healthy tissues.


In the case of GPA, the blood vessels are damaged. However, no type of bacteria, virus or fungus has been definitively related to the disease.


You can get this disease at any age, but it is more common in people 40 to 65 years old.


How common is it?


GPA is a very rare disease. According to the National Library of Medicine of EE. UU., Only 3 of every 100,000 people in the United States will get it.


How is it diagnosed?


Your doctor will first ask about your symptoms and medical history. Then you will have an exam.


There are several types of tests that your doctor could use to help you make a diagnosis.


Blood and urine tests


Your doctor can use any of the following blood and urine tests:




  • Antineutrophil cytoplasmic antibody test (ANCA). This blood test looks for proteins called antibodies that most people with GPA have. However, you can not definitely confirm that you have GPA. About 20 percent of people with GPA have an ANCA negative test result.


  • C-reactive protein and erythrocyte sedimentation rate (thirst rate). These blood tests can be used to identify inflammation in your body.


  • Complete blood count (CBC). A CBC is a common test that measures your blood cell counts. A low red blood cell count is a sign of anemia, which is common in people with GPA whose kidneys are affected.


  • Creatinine in urine or blood. These tests measure the levels of the creatinine waste product in your urine or blood. A high creatinine level is a sign that your kidneys are not working well enough to filter waste from your blood.


Image tests


These tests take pictures from inside your body to detect organ damage:




  • X-rays. A chest x-ray uses small amounts of radiation to take pictures of the affected area, such as the lungs and blood vessels.


  • Computed tomography. This test uses computers and rotating X-ray machines to take more detailed images of the affected area.


  • Magnetic resonance An MRI uses magnets and radio waves to produce detailed and transverse images of the area in question without bones obstructing the view of tissues and organs.


Biopsy


The only way to confirm that you have GPA is with a biopsy. During this surgical procedure, your doctor removes a small sample of tissue from an affected organ, such as your lung or kidney, and sends it to a laboratory. A laboratory technician examines the sample with a microscope to see if it looks like a GPA.


A biopsy is an invasive procedure. Your doctor may recommend a biopsy if the blood, urine or results of the imaging tests are abnormal and you suspect a GPA.


How is it treated?


GPA can permanently damage organs, but it is treatable. You may need to continue taking long-term medications to prevent the disease from coming back.


The medications your doctor can prescribe include:



  • anti-inflammatory medications, such as corticosteroids (prednisone)

  • immunosuppressant drugs, such as cyclophosphamide, azathioprine (Azasan, Imuran) and methotrexate

  • the chemotherapy drug rituximab (Rituxan)


Your doctor may combine medications such as cyclophosphamide and prednisone to reduce inflammation more effectively. More than 90 percent of people improve with this treatment.


If your GPA is not serious, your doctor may recommend treating it with prednisone and methotrexate. These medications have fewer side effects than cyclophosphamide and prednisone.


The medications used to treat GPA can cause side effects. Some side effects are serious. For example, they can lower your body's ability to fight infections or weaken your bones. Your doctor should check it for side effects like these.


If the disease is affecting your lungs, your doctor may prescribe a combination of antibiotics, such as sulfamethoxazole-trimpetoprim (Bactrim, Septra), to prevent infection.


Are there possible complications?


GPA can be very serious if left untreated, and can get worse quickly. Possible complications include:



  • renal insufficiency

  • lung failure

  • hearing loss

  • heart disease

  • anemia

  • scars of the skin

  • damage to the nose





  • deep vein thrombosis (DVT), a blood clot in the deep vein of the leg


You should continue to take your medications to avoid a relapse. The GPA returns in about half of the people within two years after stopping treatment.


What is the perspective?


The prognosis for people with GPA depends on how serious your disease is and what organs are involved. The medication can effectively treat this condition. However, relapses are common. You should continue to see your doctor for follow-up tests to make sure that the GPA does not come back and to avoid complications.



Reference: https://www.healthline.com/health/granulomatosis-with-polyangiitis






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