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Patient Education

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of rare and potentially life-threatening autoimmune diseases that affect small to medium blood vessels in the body. It is caused by the abnormal production of antibodies called ANCAs that attack and damage blood vessels, leading to inflammation and tissue damage.^1-3

ANCA-associated vasculitis includes 3 main subtypes: granulomatosis with polyangiitis (GPA; formerly known as Wegener granulomatosis), eosinophilic granulomatosis with polyangiitis (EGPA; previously known as Churg-Strauss syndrome), and microscopic polyangiitis (MPA). Patients may also be diagnosed with drug-induced vasculitis and renal-limited vasculitis.²

Patients with AAV require ongoing management to control their symptoms and prevent complications deriving from the disease or its treatment. Patient education is an essential component of this management, as it empowers patients to take an active role in their care and make informed decisions about their treatment. Patient education can increase patients’ awareness of symptoms and signs of relapse while promoting compliance with treatment.^2,3

Managing Symptoms

Symptom management is an important part of AAV patient education. In AAV, many different organs are affected, including the kidneys, stomach, intestine, and lungs. Skin lesions, such as purpura and urticaria, can also be observed.¹ Symptoms of vasculitis include fatigue, muscle and joint pain, fever, cough, hemoptysis, abdominal pain, blood in the urine, weakness, inflammation of the eyes, and numbness in the hands or feet.^2,4 Patients also report general symptoms such as weight loss, fever and sweats, and fatigue.⁴

Patients may experience flares of the disease once it is treated and in remission. Triggers for these relapses are not known, though infections and stress have been suggested to be potential factors.⁵

Read more about AAV signs and symptoms

Treatment Options

The treatment of AAV aims to induce remission to avoid or slow organ damage and then maintain remission or treat relapse.² Patient education about treatment options and possible side effects is essential, as patients may require a combination of medications to manage their symptoms and prevent complications. These medications include immunosuppressants and corticosteroids, such as Cytoxan® (cyclophosphamide), Rituxan® (rituximab), and prednisone.^2,4 These medications may, however, induce various side effects.

Common side effects of Cytoxan include⁴:

Bone marrow suppression
Increased susceptibility to infections
Nausea and vomiting
Blood in the urine due to bladder inflammation

Rituxan may lead to side effects that include⁴:

Infusion reactions
Increased susceptibility to infections
Nausea
Rash
Decreased levels of blood cells

Corticosteroids also have the potential to cause a variety of side effects, including⁴:

Gastrointestinal issues (indigestion and ulcers)
Musculoskeletal issues (loss of bone strength)
Diabetes
Increased susceptibility to infections
Weight gain
Mood changes

In addition, some of these medications, such as Rituxan, may condition the response to certain vaccinations, and patients may therefore need to vaccinate against different agents and conditions such as pneumococcus, influenza, hepatitis B, and tetanus.⁴ Live-attenuated vaccinations, such as the yellow fever and varicella/herpes zoster vaccines, should be administered at least 6 months after the last course of Rituxan.⁴

Read more about AAV treatment

Follow-Up Care and Self Care

Regular follow-up care is important for managing AAV and preventing complications. Patients should therefore attend regular check-ups with their health care provider.⁵ Monitoring of the disease can involve regular blood tests, chest x-rays to examine the heart, lungs, and vasculature, and cardiac testing such as echocardiography or electrocardiography.⁵

Self care and lifestyle changes are also essential for managing AAV and preventing complications. Maintaining a well-balanced and healthy diet is important and can be achieved with the help of a dietitian. Patients with high blood pressure may require a low-salt diet, while patients with renal insufficiency will need to control protein and potassium intake. Patients with diabetes will need to follow specific dietary recommendations, as the intake of glucocorticoids may have an impact on their condition.⁵

Read more about AAV diet and nutrition

Patient Support and Advocacy

Patients with AAV may benefit from joining support groups or advocacy organizations, as these groups can provide valuable resources and emotional support. Through these groups and organizations, patients with AAV can be encouraged to connect with other patients and families who are experiencing similar challenges. The Vasculitis Foundation is an organization that promotes education, research, and clinical awareness of the disease. They also provide virtual support groups and access to resources, such as support and wellness programs, patient advocacy groups, and clinical trials.⁶

Read more about AAV care team

References

1. ANCA-associated vasculitis. Genetic and Rare Diseases Information Center (GARD). Updated February 2023. Accessed March 9, 2023.

2. Qasim A, Patel JB. ANCA positive vasculitis. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2022. Updated May 29, 2022. Accessed March 9, 2023.

3. Brolin S, Welin E, Lövström B, Bruchfeld A, Gunnarsson I, Pettersson S. Exploring the educational needs of patients with systemic vasculitis using the educational needs assessment tool. Rheumatol Adv Pract. 2022;6(2):rkac062. doi:10.1093/rap/rkac062

4. ANCA-associated vasculitis: information for patients from the Renal Department. East Kent Hospitals University NHS Foundation Trust. May 2021. Accessed March 9, 2023.

5. Living with vasculitis. National Heart, Lung, and Blood Institute. Updated March 24, 2022. Accessed March 9, 2023.

6. Vasculitis Foundation homepage. Vasculitis Foundation. Accessed March 9, 2023.

Reviewed by Kyle Habet, MD, on 3/21/2023.

Diana Diez Gaspar, PharmD, PhD

Diana earned her PhD and PharmD with distinction in the field of Medicinal and Pharmaceutical Chemistry at the Universidade do Porto. She is an accomplished oncology scientist with 10+ years of experience in developing and managing R&D projects and research staff directed to the development of small proteins fit for medical use.

ANCA-Associated Vasculitis (AAV)