Gain the knowledge and tools you need to navigate your personal journey with ANCA-associated vasculitis (GPA or MPA).
Receive helpful disease and treatment information, access a Doctor Discussion Guide, and request a hospital emergency readiness kit.
Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis describes a group of rare diseases that cause inflammation in small- to medium-sized blood vessels in your body. This inflammation can reduce blood flow, impacting the organs and how they work. The exact cause of ANCA-associated vasculitis is currently unknown, but it is thought to be a combination of genetic and environmental factors.
Granulomatosis with polyangiitis (GPA)
GPA is a disease resulting from inflammation of the blood vessels. In GPA, immune cells can cluster to form what is called a granuloma. The lungs, kidney, sinuses, ears, nose, and throat are often affected.
Microscopic polyangiitis (MPA)
MPA shares similarities with GPA, but granulomas don't form. The impact on the kidneys may be more severe, and people with MPA may be more likely to have bleeding in their lungs.
Some terms you may hear when describing these diseases are: severe and active.
“Severe disease” means that you have signs and/or symptoms of ANCA-associated vasculitis that could be organ- or life-threatening.
“Active disease” means that you have signs and/or symptoms of the disease that are new, not getting better, or getting worse.
About 80-90% of patients with ANCA-associated vasculitis have kidney- or other organ-threatening signs and symptoms, which can be considered severe active disease
GPA and MPA can impact many different parts of your body. This diagram can help you understand some of the signs and symptoms of GPA and MPA. For ~50% of people,* even in remission,† vasculitis symptoms are debilitating, so it is important to keep track of signs and symptoms and discuss them with your doctor. Keeping a notebook or journal can help you have an organized conversation with your doctors to take control of your treatment.
Additionally, signs and/or symptoms can be persistent. While relapse is common with GPA and MPA, it is often associated with significant harm.
If you notice any of these symptoms, it is important to tell your doctor and discuss which treatments are available to help you manage them. Please note these are just some of the signs and symptoms of GPA or MPA. Severe active GPA or MPA can present differently in each patient.
According to an online, self-administered survey of 100 patients with GPA or MPA from July 21-August 25, 2022.
Remission is the lack of GPA or MPA symptoms on or off immunosuppressive therapy.
When managing severe active GPA or MPA, you and your doctor may face challenges with the disease as well as the medications you may be taking to treat it.
It can often feel difficult or overwhelming managing severe active GPA or MPA. It is possible to address these challenges with your doctor to help manage your GPA or MPA.
Click on each of the challenges below to learn more about managing severe active GPA or MPA, and the importance of talking about these with your doctor.
Click on each of the challenges below to learn more about managing severe active GPA or MPA, and the importance of talking about these with your doctor.
Since GPA and MPA share many signs and symptoms with other diseases, diagnosing them can be tricky. However, early diagnosis is extremely important to coming up with a treatment plan that can help manage these conditions.
There are a number of ways your doctor will try to confirm a potential GPA or MPA diagnosis:
Depending on how GPA or MPA has impacted your body, you may need to see different types of doctors, such as a rheumatologist or nephrologist, to help manage your symptoms.
Once a diagnosis of GPA or MPA is suspected, a tissue sample of the affected area known as a biopsy may be performed to confirm the presence of vasculitis. Treatment should not necessarily be delayed for a biopsy. However, biopsies can be helpful when trying to understand if an organ is impacted by GPA or MPA.
The right treatment may make a difference in GPA or MPA
To help control disease activity, your doctor may prescribe immunosuppressants, such as glucocorticoids.
Immunosuppressants
Immunosuppressants are medications that target inflammation by lowering the activity of the body’s immune system and can be used to to help you reach remission.
Glucocorticoids
Glucocorticoids (also called steroids), such as prednisone, are one type of immunosuppressant. While glucocorticoids can be helpful in controlling disease activity, they can also cause serious side effects. This is especially true if used over a long period. To help, doctors are shifting to treatments that can reduce the time a person takes high-dose glucocorticoids.
There are several treatment options available for patients with GPA and MPA. It is important to talk to your doctor about what treatment options may be right for you.
“For someone who is newly diagnosed…ask a lot of questions. Fight for yourself because you’ve got to take care of your whole being. Living a healthy life as a rare disease patient encompasses all facets of life.”
– Brandon, person living with GPA
Have an open conversation
Open conversation between you and your doctor is very important because you both may have different expectations of what successful treatment looks like. Be sure to have open communication on any questions or concerns you may have and let them know of any changes in your health that you may be experiencing.
Below are some questions that can help you and your doctor talk about important topics when it comes to your treatment and expectations.
Prepare for your appointment
It is important to be organized and use your time wisely with your doctor. Some tips to consider:
“For someone who is newly diagnosed…ask a lot of questions. Fight for yourself because you’ve got to take care of your whole being. Living a healthy life as a rare disease patient encompasses all facets of life.”
– Brandon, person living with GPA
Connecting with people like you can also provide a sense of community and support. The following organizations provide education, support, and resources that may help you throughout your journey.
These organizations are not owned or controlled by Amgen, and Amgen is not responsible for their content. However, you may find them helpful.
The Vasculitis Foundation supports, inspires, and empowers people and families living with vasculitis through a wide range of education, research, clinical, and awareness initiatives.
The ERDC (Eosinophilic & Rare Disease Cooperative) is dedicated to helping improve access to care, treatment and improved outcomes for people with rare disease.
The American Kidney Fund (AKF) works on behalf of Americans living with and at risk of kidney disease with programs that support people from prevention through transplant and beyond.
NORD (National Organization for Rare Disorders) is a patient advocacy organization committed to the identification, treatment, and cure of rare disorders through programs of education, advocacy, research, and patient services.
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Recognizing the signs and symptoms of severe active GPA or MPA at an early stage can lead to a timely diagnosis. An early diagnosis allows you and your doctor to work together to decide on a plan to help manage your condition.
However, about 1/3 of people experience a significant diagnostic delay of more than 6 months.
The goal of treatment is to achieve and sustain remission. Remission is the lack of GPA or MPA signs or symptoms on or off immunosuppressive therapy. Response to initial treatment for severe active GPA or MPA varies and some people do not achieve remission.
It is critical to keep track of your symptoms and tell your doctor when you feel like they may be coming back. This can help you and your doctor decide on the best way to manage your GPA or MPA to prevent relapse.
GPA and MPA are chronic diseases that can relapse. This means that while there may be times where it seems like your symptoms are getting worse, there may also be times where symptoms seem to get better as well.
Relapse is when your GPA or MPA symptoms return after being previously controlled. Preventing relapse is key to staying in remission.
Steroids, called glucocorticoids, are commonly used for GPA and MPA treatment, but they can have side effects.
AAV could lead to impacted organ function, especially in the lungs and kidneys.
AAV threatens the kidneys or another organ in about 80% - 90% of cases, which can be considered severe active disease.
“Quality of Life” describes how well you’re doing and feeling day-to-day. If your treatment plan is working for you, it can improve your quality of life.
Only about 1/4 of people are satisfied with their medication’s ability to control symptoms while maintaining a good quality of life.*
*According to an online, self-administered survey of 100 patients with GPA or MPA from July 21-August 25, 2022.
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This link goes to the website of a treatment option for adults with severe active GPA or MPA.
The link goes to the website of a treatment option for severe active GPA or MPA. The information contained in this site is intended for U.S. healthcare professionals only.
