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Granulomatosis with polyangiitis (GPA)

Granulomatosis with polyangiitis (GPA, formerly called Wegener’s granulomatosis)

Although the disease can involve any organ system, it most commonly affects the upper respiratory tract (sinuses, nose, ears), lungs, kidneys, skin and nerves.

What are early signs of GPA?

The first signs of GPA can be vague and may include fever, loss of appetite, weight loss and fatigue. Almost always there are symptoms related to the upper respiratory tract such as sinus pain, runny nose, bloody fluid from the nose, crusting in the nose, and reduced hearing. A common sign of the disease is the lack of improvement and progressive worsening of these symptoms after multiple courses of antibiotics.

The lungs are also commonly affected. Symptoms can include a cough with or without bloody phlegm, shortness of breath, and chest discomfort.

Kidney involvement can present with red, brown or foamy urine, high blood pressure or swelling in the feet and ankles.

Muscle and joint pain or, occasionally, joint swelling are also common.

Involvement of the skin is quite common – and at times will be the first symptom identified. Skin lesions often appear as small red or purple raised areas or blister-like ulcers or nodules that may or may not be painful.

Some patients experience eye problems. These can include pain, redness, altered vision and protrusion of the eyeball.

How is GPA diagnosed?

GPA can be challenging to diagnose, as it presents with vague symptoms. A thorough history, physical exam, blood work, imaging and biopsies are often required to make a diagnosis.

Blood tests can detect inflammation in the body (e.g. C-reactive protein or erythrocyte sedimentation rate). Four out of five patients will test positive for anti-neutrophil cytoplasmic antibodies (ANCA). Patients can often have anemia (low red blood cells), especially with high levels of inflammation in the body. A urine test should be done to check kidney function, by looking for blood and protein in the urine.

Imaging can be useful for diagnosis, depending on what area of the body is involved. For example, if the patient has respiratory symptoms, a chest x-ray or computerized tomography (CT) scan may be required.

Biopsy: The most accurate way to diagnose GPA is though a biopsy. For this, a small sample of tissue is taken from the affected organ.

What are the risk factors for GPA?

The cause of GPA is not known. GPA is an auto-immune disease in which the body's own immune system attacks the blood vessels and other tissues. Genetics appear to play a small role, and it is very unusual for GPA to occur twice in the same family. It is thought that infections may contribute as a trigger for GPA, but no specific infection has yet been identified.

See your doctor if you have a persistent runny nose that doesn’t respond to over-the-counter medications, especially if you also have nosebleeds and pus, are coughing up blood, or have other warning signs of granulomatosis with polyangiitis.

This disease can get worse quickly, so getting diagnosed quickly and onto a treatment plan is very important.

How common is GPA?

GPA is relatively rare, affecting roughly 3 out of every 100,000 Canadians. It strikes men and women equally. It typically occurs in the middle age, but is found in people of all ages.

With appropriate treatment, the outlook is good for people with GPA, and it is not unusual for people to see their symptoms disappear completely. However, about half of all people with the disease may require long-term use of medications to keep the disease in remission. The disease may flare frequently within two years of stopping medication but can also occur during treatment. For this reason, it is very important that people with GPA continue to see their doctors regularly, even if they have felt well for several years.

Medications

Medications used to treat GPA are designed to control the disease, slow its progression, and to prevent flares.

These medications can be very complex, so you are encouraged to ask for in-depth explanations from your health care team – including pharmacists, who are excellent sources of information. Some medications used to treat GPA include prednisone, cyclophosphamide, rituximab, methotrexate, azathioprine, and mycophenolate mofetil. There are two stages of treatment: the induction phase, during which stronger medications are used to put the disease into remission, and the maintenance phase, in which medications are used to keep the disease under control.

To explore these and other medical treatments, The Arthritis Society has developed a comprehensive expert guide that delivers detailed information on medications used to treat arthritis, including GPA.

EXPLORE: Arthritis Medications – A Reference Guide

The optimal treatment is what is best in each individual case – so speak with your specialist about what kind of medications are most appropriate for you for several features of the disease. Your primary care physician or nurse practitioner and pharmacist may also be helpful.

Therapies

These healthcare professions have advanced training from a university and are registered to practice by their provincial/territorial association.

A physiotherapist (PT) can develop an individualized program designed to help you increase your strength, flexibility, range-of-motion, and general mobility and exercise tolerance through a wide variety of therapeutic treatments and strategies. These include exercise prescription, physical interventions, and relaxation, in addition to advising you on other techniques for reducing pain and increasing your overall quality of life. PTs can also refer you to other health professionals and community services for further measures that will help you adapt to your changing circumstances.

An occupational therapist (OT) trained in arthritis management can analyze everything you do in a day and develop a program to help you protect your body and minimize fatigue. If necessary, your OT can help you redesign your home or workplace to make it easier for you to work or simply get around. They can also make or recommend a number of different splints, braces, orthopedic shoes and other aids that can help reduce your pain and increase your mobility and functionality. Their goal is to prepare you, using assistive devices and adaptive strategies, to reclaim as much of your former life as possible.

Getting diagnosed with GPA can be quite stressful. It is very important for you to maintain a strong support system. Talking to family and friends can be very helpful. Also, there might be a GPA support group in your community. You can contact Vasculitis Foundation Canada for more information.

Since GPA is complex, the more you understand the disease, the better you are prepared to deal with the symptoms, complications and side effects of medications.

In addition to following your treatment plan, there is a lot you can do yourself to help decrease your pain and increase your movement. Physiotherapy, occupational therapy, regular exercise and relaxation techniques are very important parts of your overall treatment plan. Although you can undertake many of these activities on your own, it is important to assemble a health-care team who can help oversee and direct your self-management efforts. Learning as much as you can about the disease is also important to help you make informed decisions about treatment and lifestyle, and maximize your quality of life.

In some cases, people with GPA also experience joint pain or restricted mobility. If you are experiencing joint pain, check out our self-management resources.

Living well with arthritis

There is a lot you can do to take control and actively manage your arthritis. Below we have listed a few resources to help you learn more about actively managing your arthritis to live better.

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Contributor(s)

This information was last updated September 2017, with expert advice from:

Dr. Natasha Dehghan, MD, FRCPC
Clinical Assistant Professor, Division of Rheumatology, University of British Columbia
Director, Vasculitis Clinic, Mary Pack Arthritis Centre (Vancouver, BC

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