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Diseases > Granulomatosis with Polyangiitis (GPA) > What can I do about it?

What can I do about Granulomatosis with Polyangiitis?

Granulomatosis with polyangiitis is a very serious disease. Before the advent of modern therapies, people with granulomatosis with polyangiitis often died within a few months to years. Luckily, that’s not the picture today. There are effective treatments available. Even if they don’t cure granulomatosis with polyangiitis, they can make living with the condition much more comfortable.

If you have granulomatosis with polyangiitis or think you may have it, your family doctor should refer you to a specialist immediately. This is a very serious illness that should be treated early and aggressively. A rheumatologist is one of the best people to help you manage your condition. Many people with inflammatory types of arthritis who see their rheumatologist regularly benefit from the highest level of care. Some people will need to see kidney specialists (nephrologists), lung specialists (respirologists), or nerve specialists (neurologists).

Here are some recommendations:

  • Learn as much as you can about this disease. Education is very powerful and we’ve aimed to develop this RheumInfo website to be accessible and easy to understand for everyday people living with granulomatosis with polyangiitis and other forms of inflammatory arthritis
  • Attend your rheumatologist appointments regularly
  • Get your blood tests done as suggested by your rheumatologist
  • Learn about the medications used to treat granulomatosis with polyangiitis. The RheumInfo website has many interactive and valuable tools to help you understand these medications and their impact on your disease

Treatment of Granulomatosis with Polyangiitis

Now is probably the best time in history to get granulomatosis with polyangiitis. The approach to treatment has changed and the medicines available today are much more effective than in the past.

People with granulomatosis with polyangiitis can lead active and productive lives with the right kinds of treatment. Whatever treatment approach you choose it is essential to remember two key points: treat granulomatosis with polyangiitis early and treat it aggressively. Don’t wait. There is great treatment available that can help get you back to leading a full life.

Why is it important to treat Granulomatosis with Polyangiitis early?

Research has shown us if you treat granulomatosis with polyangiitis early you are much more likely to get into remission. Even a few months can make a difference. Here’s an analogy. Imagine you are sitting in your living room enjoying a nice cup of coffee. You look over to the kitchen and see a fire burning on the stove. What do you think you’ll do? One option is to just sit there and wait until the fire gets worse and spreads to the walls or the ceiling. The second option is to grab the phone, dial the fire department, and grab the fire extinguisher.

You can think of granulomatosis with polyangiitis like a fire in your small blood vessels and organs. You want to get that fire put out as quickly as possible so it doesn’t damage your body. Once the damage from granulomatosis with polyangiitis is done it cannot be reversed. We want to stop granulomatosis with polyangiitis before it damages your blood vessels and tissues.

Why is it important to treat Granulomatosis with Polyangiitis aggressively?

Using the same fire analogy, we’ve decided to call in the fire department. Now we need to make sure we have the right tools to put out the fire. We don’t want a bucket and water. We want a fire truck with a big hose. We might even want more than one fire truck. The faster we can get that fire out the better things will be in the long run.

Medications for Granulomatosis with Polyangiitis

Medications for granulomatosis with polyangiitis fall into two broad groups. The first includes medications that are used initially to bring the disease under control quickly. These are called induction therapies. The second group of medications control the disease over the long-term and are called maintenance therapies.

Induction Therapies

Corticosteroids (prednisone), cyclophosphamide and rituximab are medications that help to rapidly reduce the inflammation and control the symptoms associated with granulomatosis with polyangiitis. Once the disease is in remission (when there are no more symptoms and markers of inflammation are back to normal), induction therapies are often tapered. This means the dose is reduced and sometimes stopped altogether.

Maintenance therapies

Azathioprine, mycophenolate mofetil or mycophenolic acid, and methotrexate are medications that are typically used for maintenance (long-term) therapy. These medications usually take longer to begin to work. Maintenance medications might be added on to the induction therapy initially, but unlike induction therapies, maintenance medications are usually continued over the long-term to keep the disease in remission.

For more information about specific medications used to treat granulomatosis with polyangiitis, refer to the “pictopamphlets” in the Medications section of this website.

Corticosteroids (prednisone)

Medications like prednisone help control inflammation. They may also prevent long-term damage in some people. Prednisone is a very effective medication to control the inflammation of granulomatosis with polyangiitis. In the short-term, prednisone may be given by intravenous infusion in the hospital. After a few days, this is usually switched to oral prednisone. As symptoms improve and markers of inflammation return to normal, the dose of prednisone can be gradually reduced (tapered).

When used for long periods of time, prednisone can have side effects. You should to discuss the risks and benefits of using prednisone with your doctor. Some patients who have joint involvement also benefit from cortisone injections directly into a joint. This should be discussed with your rheumatologist.

Cyclophosphamide

A medication called cyclophosphamide is often used with prednisone to get the disease under control. Cyclophosphamide can be given by mouth or by intravenous infusion. If there is very serious lung, kidney or nerve involvement, an intravenous infusion may be easier to administer.  Cyclophosphamide can increase your risk of infection (be careful with fevers). Cyclophosphamide can also affect your ability to have children (fertility) – this needs to be discussed with your doctor.

Rituximab

This is a newer type of therapy called a “biologic” medicine. Rituximab has been shown to be as effective as cyclophosphamide and is now approved for the treatment of granulomatosis with polyangiitis. Although it is more expensive, it may be appealing to women of childbearing age because it does not affect fertility.

DMARDs

The Disease Modifying Anti-Rheumatic Drugs (DMARDs) are medications that are typically used to treat rheumatoid arthritis. Examples that have been shown to be effective in people with granulomatosis with polyangiitis include azathioprine (Imuran), methotrexate, and mycophenolate mofetil or mycophenolic acid. In many cases the DMARDs are used for maintenance therapy once the disease is under control. In milder cases of granulomatosis with polyangiitis they may be used to induce remission.

Most DMARDs take about 6-12 weeks before they begin to work. Some people might not feel any effect when they first start taking DMARDs. Even if this happens, it’s important to keep taking DMARDs to help keep inflammation under control and to maintain remission.

Exercises for Granulomatosis with Polyangiitis

Exercise is important to overall health. Regular physical activity can also help you deal with fatigue. The level and amount of exercise you can do depends on the extent and activity of your granulomatosis with polyangiitis. A good rule of thumb is to aim for 20-30 minutes of physical activity every day.

Aerobic exercise such as walking, swimming or bicycling works your heart and increases your overall fitness. It is also an important part of keeping a healthy weight. A trained arthritis physiotherapist can help design an exercise program tailored to you and your needs. They can also help you choose mobility aids and teach you how to protect your joints if you have joint involvement.

Below are some useful articles on exercising with arthritis – many of the tips can also be applied to people with granulomatosis with polyangiitis:

Exercise and Arthritis: An article by arthritis physiotherapist, Marlene Thompson

Exercising in a Flare: Another excellent article written by Marlene Thompson on how to cope with flares through your exercise routine.

Natural or Home Remedies for Granulomatosis with Polyangiitis

There are no known natural remedies or complementary therapies that have been proven to help granulomatosis with polyangiitis in any significant way. However, it’s important to check with your rheumatologist to make sure that nothing interacts with your medication if you choose to use natural remedies or complementary therapies.

GPA is a very serious disease that can result in death. It is important to use therapies that are proven to work. Natural therapies are not proven to work for GPA.

Diet for Granulomatosis with Polyangiitis

Questions about diet and granulomatosis with polyangiitis are very common. We all want to know what we can do to help ourselves. Can we change our diet to improve our immune system and help our condition? Changing our diet gives us a sense of control over a disease which often seems to have a mind of its own.

Unfortunately, there is no diet that has been proven to significantly alter the course of granulomatosis with polyangiitis or other types of inflammatory arthritis. Following the basics of healthy eating can help improve health and well-being in everyone, including those with granulomatosis with polyangiitis. Eating well can also help you keep a healthy weight and reduce your risk of diabetes, which can be associated with long-term use of prednisone.

Alcohol and Granulomatosis with Polyangiitis

Many of us like to share a glass of wine, a beer, or a spirit from time to time. Unfortunately, due to the nature of granulomatosis with polyangiitis, some people may turn to alcohol to help cope with the distress. Alcoholic beverages are not an effective treatment for granulomatosis with polyangiitis. They can also interact with some medications. Examples include the DMARDs methotrexate or azathioprine.

Smoking and Granulomatosis with Polyangiitis

Cigarette smoking, whether you have granulomatosis with polyangiitis or not, has no positive effects on any aspect of your health. Smoking can also worsen the symptoms of granulomatosis with polyangiitis by breathing toxic chemicals into airways that are already affected by the disease.

So if you are a smoker with granulomatosis with polyangiitis, quitting could be one of the best things you can do to improve your overall health.

Concluding Thoughts

Granulomatosis with polyangiitis is a very serious disease. It is very important to treat this disease early and aggressively to get the inflammation under control.