Description

Granulomatosis with polyangiitis (GPA) is a rare disease that causes swelling, also called the inflammation of the small blood vessels. This condition primarily affects the blood vessels of the nose, sinuses, throat, lungs, and kidneys. But it may affect any organ.

PAM was called Wegener granulomatosis. It is one of a group of diseases of the blood vessels, called vasculitis. GPA slows down the blood flow to some organs. The affected tissue may develop swollen areas called granulomas. Granulomas can cause damage to these organs, and affect the way in which we work.

The symptoms of GPA vary. At first, the symptoms may resemble those of a cold. Other symptoms depend on which organs the disease affects. The cause of the PAM is not clear, but the immune system may play a role.

It is important for health professionals to detect and treat GPA as soon as possible. Early treatment can help people to live a full life. Without treatment, the disease can lead to organ damage that is sometimes fatal.

Symptoms

The symptoms of granulomatosis with polyangiitis (GPA) can vary widely from one person to another. The symptoms can start quickly, or it can develop over months.

In the beginning, GPA can cause general symptoms such as:

  • Fever.
  • The tired and not feeling well, also called upset.
  • The weight loss without a clear reason to do so.
  • The muscle aches and pain.
  • Stiffness of the joints.

The specific symptoms depend on which parts of the body GPA affects.

Ear, nose and throat, symptoms of

If GPA affects the ears, nose, or throat, symptoms can include:

  • Purulent drainage with scabs in the nose.
  • Sores in the nose or the mouth.
  • Congestion.
  • Infections of the sinuses.
  • Nosebleeds.
  • Earaches or fluid that drains from the ear.
  • The inflammation of the cartilage of the ears, or on the bridge of the nose.

Lung and trachea symptoms

If GPA affects the lungs or trachea, the symptoms may include:

  • Coughing, at times with phlegm with blood.
  • Shortness of breath or wheezing.
  • A high-pitched breathing sound called stridor.
  • The hoarse voice.

For some people, the disease only affects the lungs. Without treatment, GPA, can lead to pulmonary hemorrhage and scarring over time.

Kidney symptoms

If GPA affects the kidneys, you may not have symptoms. Or you might have symptoms such as:

  • The high blood pressure.
  • The swelling of the legs.
  • Blood in the urine.

The symptoms of the eye

If GPA affects the eyes, the symptoms may include:

  • Redness of the eyes.
  • Burning in the eyes, or pain.
  • The feeling that something is caught in the eye.
  • Blurred vision or double vision.
  • The bulging of the eyes.
  • Swelling of the eyelids.

Other symptoms

Other symptoms of GPA may include:

  • The loss of sensation, tingling, or deep pain in the limbs, fingers, or toes of the feet due to nerve damage.
  • Eruptions in the skin, such as reddish spots or stains may be more difficult to view in Black or brown skin.
  • Digestive problems, including stomach pain, diarrhea and bloody stool.

When to see a doctor

GPA symptoms can appear similar to those of some other health problems. The first symptoms may feel like a cold or the flu and may last for weeks or more. Consult your health care professional if you have a runny nose that does not improve with medications for the common cold. Be sure to get a health checkup if you have a runny nose, along with:

  • Bleeding from the nose and pus-like fluid.
  • Bloody cough.
  • Other symptoms of granulomatosis with polyangiitis.

The disease can get worse quickly. Without treatment, it can lead to other serious health problems called complications. That is why it is important for a health professional to find and to start treatment early.

Causes

The exact cause of granulomatosis with polyangiitis (GPA) is not clear. The immune system may play a role. Some white blood cells can't function as they should, and they may be involved in the disease. The immune system makes proteins called antibodies that protect the body from microorganisms, such as viruses and bacteria. GPA has been related to the presence of certain antibodies that attack healthy cells by mistake.

The condition can lead to swollen, narrowing of the blood vessels and harmful, the inflamed tissue masses called granulomas. Granulomas can destroy healthy tissue. And narrowing of the blood vessels to reduce the amount of blood and oxygen that reaches the tissues and organs.

PAM is not transmitted from person to person. And it is likely that you do not pass from parents to children through genes.

Risk factors

Age is a risk factor for granulomatosis with polyangiitis. Most often, the disease affects people between the ages of 40 and 65 years. But it can occur at any age.

Complications

Granulomatosis with polyangiitis (GPA) can lead to other health problems called complications. Complications depend on the organs or parts of the body the disease affects. These may include:

  • The hearing loss.
  • A loss of height at the bridge of the nose caused by the cartilage weakened.
  • Sores on the skin or scars.
  • The kidney damage or kidney failure.
  • The bleeding and the formation of scar tissue in the lungs.
  • A blood clot that forms in one or more of the deep veins, usually in the leg.
  • Heart disease, such as inflammation of the sac that surrounds the heart.
  • Rarely, stroke or loss of vision.

Diagnosis

The diagnosis involves the steps that your health care team takes to find out if you have granulomatosis with polyangiitis (GPA). Your healthcare provider will ask about your symptoms and medical history. You can also get a physical exam. You may need other tests.

Laboratory tests

Blood tests can check for:

  • The signs of inflammation that GPA causes. A high level of a protein produced by the liver, so-called C-reactive protein, may be a clue to the inflammation. So can red blood cells that rapidly sink to the bottom of a test tube. This measure is known as a high erythrocyte sedimentation rate, also called a high rate of sedimentation.
  • Anti-neutrophil cytoplasmic antibodies. These proteins of the immune system to attack healthy white blood cells called neutrophils by mistake. The antibodies appear in the blood of the majority of people who have GPA.
  • Low levels of red blood cells to carry oxygen. This is called anemia, and it is common in people with GPA.
  • The symptoms of the kidneys aren't properly filtering waste products from the blood.

The analysis of urine can reveal if the urine for red blood cells or protein in excess. These could mean that the disease is affecting the kidneys.

Imaging tests

If you have pulmonary symptoms, chest x-rays and ct scans can help you find out if granulomatosis with polyangiitis is the cause. The CT scan uses X-rays and a computer to make more detailed images of the internal organs of the body. The CT scan also can help you find out if granulomatosis with polyangiitis is the cause of head or neck symptoms. Once a person starts treatment for GPA, health professionals can use the CT scan to find out if the treatment is working.

Biopsy

During a biopsy, a healthcare provider removes a small sample of tissue from the affected area of the body. For example, a sample of tissue from the lungs, the skin, the kidneys, or in the inside of the nose. The tissue sample is examined with a microscope for signs of inflammation or damage. This test can confirm if you have granulomatosis with polyangiitis.

Treatment

The treatment for granulomatosis with polyangiitis (GPA) includes medications to get the disease under control and keep it from coming back. Along with getting the attention of your health care professional, it is likely that you will need treatment and follow-up care of several specialists. The specialists that you need to depend on the organs affected. With early treatment, many people who have GPA to get better and lead a full life.

Drugs

Medicines called corticosteroids help to reduce the response of the immune system and reduce inflammation of the blood vessels. Common side effects include weight gain, risk of infection and thinning of the bones.

Other medications that decrease the immune system response include:

  • Rituximab (Rituxan).
  • Azathioprine (Azasan, Imuran).
  • Mycophenolate (CellCept, Myhibbin).
  • Methotrexate (Trexall).
  • Cyclophosphamide.
  • Avacopan (Tavneos).

Once your condition is under control, you can stay in some of the long-term medications. You can listen to your health care provider call this a maintenance therapy. The long-term goal of treatment is to prevent the GPA becomes, also called relapse. Drugs that can be used long-term include rituximab, methotrexate, azathioprine and mycophenolate.

Drugs as these may increase the risk of infection. Cyclophosphamide can cause stomach upset, diarrhea, and loss of hair. Your healthcare provider may prescribe other medications to help prevent the side effects of the prescribed treatments.

Plasma exchange

Plasma exchange removes the liquid portion of blood, called plasma. In the majority of people with GPA, the plasma proteins that are linked with the disease. During plasma exchange, you will receive fresh plasma or a protein produced by the liver called albumin. Albumin helps the body make new plasma. Sometimes, the plasma exchange is used to treat people who have very severe GPA. You can help the kidneys get better. Another name for this treatment is plasmapheresis.

Coping and support

It is likely that improved after treatment for granulomatosis with polyangiitis (GPA). Even so, you could feel the tension on the disease comes back or the damage it can cause. Here are some tips to help you cope:

  • Understand your condition. Learn all you can about the GPA. Knowledge can help you to deal with the complications, medication side effects and relapse. Talk with your health care professional. You can also speak with a counselor or a social worker medical.
  • Build a strong support system. Family and friends can help you cope. And you might find it helpful and comforting to talk with other people who are living with the disease. Ask a member of your health care team about how to connect with a support group.

Preparing for your appointment

It is likely to start by seeing your usual health professional. You may also be referred to a specialist, such as, for example:

  • A doctor called a rheumatologist that deals with conditions of the joints, muscles and the immune system.
  • A doctor called a pulmonologist who treats diseases of the lungs.
  • A doctor called otolaryngologists who treats ear, nose, throat, and conditions.
  • A kidney doctor called a nephrologist.
  • A doctor called a neurologist who treats disorders of the nervous system.

A specialist will likely be to find out if you have granulomatosis with polyangiitis.

Here's some information to help you prepare for your appointment.

What you can do

Be aware of the rules to follow prior to your professional checkup. When you make the appointment, ask if you need to do anything in advance. For example, you can limit your diet.

If you have recently had some kind of blood tests or chest X-rays to another doctor's office or in the hospital, ask the staff there to forward the test results and X-rays for the health care professional that you are watching. Or pick up the material to make sure that it arrives to your health professional.

Get a referral if your insurance company requires for visits to specialists. Make sure that you have a reference letter has been sent to health professionals or bring the letter with you.

Before your appointment, also make a list of:

  • Your symptoms and when they began.
  • Key of the personal medical information, including other recent health conditions and major stresses that he has had.
  • The drugs, vitamins, and supplements you are taking, including dosage.
  • Questions to ask your health care professional.

Have a family member or friend with you to the appointment if you can. This person can help you remember the information that you receive.

For granulomatosis with polyangiitis, questions to ask your health care team include:

  • What is likely causing my symptoms? What are other possible causes?
  • What tests are needed? How do I prepare for them?
  • Is my condition as a short-term or long-term?
  • What treatment do you recommend it?
  • There are treatment options apart from the main, the treatment that you are suggesting?
  • How long is treatment?
  • I have another medical condition. How can I better manage my conditions?
  • Do you have brochures and other printed materials to give to me? What sites do you recommend?

What to expect from your doctor

Your healthcare provider is likely to ask questions such as:

  • Do your symptoms occur often or once in a while?
  • How bad are the symptoms?
  • Have you had a fever?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?
  • Do you smoke?

What you can do in the meantime

If your symptoms worsen, inform your primary health care provider. That way, your health care professional may treat of getting to a specialist quickly.

Symptoms and treatment of Granulomatosis with polyangiitis