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ANCA-Associated Vasculitis (AAV)

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Care Team

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of rare autoimmune diseases of unknown etiology. The disease is characterized by inflammatory cell infiltration resulting in blood vessel necrosis. AAV is comprised of three main subtypes:  granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA).1 

Given the multisystemic nature of vasculitis, the involvement of interprofessional teams is crucial in providing comprehensive treatment for patients with the condition. Lung and kidney diseases are most commonly linked with AAV. The morbidity of the disease can be reduced with the involvement of rheumatologists, nephrologists, pulmonologists, pharmacologists, and nurses in patient care.2

Primary Care Physicians 

A primary care physician is an expert in family medicine, general internal medicine, or general pediatrics who, at the time of initial contact, renders conclusive care to the undiagnosed patient and accepts ongoing responsibility for the patient’s comprehensive care.3 

Read more about AAV patient education

Rheumatologists

A rheumatologist is an internist or pediatrician with additional training in the diagnosis and management of diseases that affect the muscles, bones, joints, ligaments, and tendons.4 

A multidisciplinary strategy is required to manage vasculitis, involving the rheumatologist and primary care physician. Delays in achieving a correct diagnosis can cause significant morbidity and higher mortality in patients with vasculitis.5 Physicians must spend more time with patients and consider the individual requirements of each patient to improve their health care outcomes. A strong, sincere engagement between patients and doctors can increase patient education and self-management.6 

Read more about AAV diagnosis

Nephrologists 

A nephrologist is a doctor who specializes in the diagnosis and management of kidney diseases. Nephrologists are also trained to help handle the effects of renal impairment on the rest of the body.7

The most frequently affected vital organs in AAV are the kidneys, and the severity of renal disease at diagnosis and its response to therapy significantly impact patient outcomes. In renal-limited AAV, organ involvement is limited to the kidneys, but all other types of AAV are multi-systemic and can also involve the upper respiratory system, lungs, and/or skin, in addition to other systems.8 

Clinical Pharmacologists

A clinical pharmacologist treats patients with general medical conditions and those who are experiencing medication-related side effects. They frequently get requests for information and assistance from general practitioners or hospital colleagues.9

It is important to inform patients taking methotrexate to abstain from alcohol and other hepatotoxic substances. The side effects of corticosteroids, such as weight gain, hirsutism, hypertension, sleep disturbances, and glucose intolerance, should be considered by patients taking them. Patients on immunosuppressive therapies must be reminded to visit a doctor if they experience any signs of infection. Birth control options should be discussed with patients of childbearing age receiving teratogenic drugs like methotrexate and warfarin.5 

Read more about AAV therapies

Pulmonologists

A pulmonologist is a physician who specializes in the respiratory system. Pulmonologists see patients whose complaints concern the lungs or other parts of the respiratory system.10 

Lung lesions are a very common and important clinical manifestation of AAV. The most common symptoms of MPA are diffuse alveolar hemorrhage and pulmonary fibrosis.11 

Read more about AAV treatment

Neurologists 

A neurologist is a medical specialist who diagnoses and treats problems with the nervous system, such as conditions that affect the nerves, spinal cord, and brain.12 Up to 30% of patients with AAV may exhibit muscular weakness, trouble walking, paresthesias like numbness, and peripheral neuropathy, which often shows up as foot drop or wrist drop.13

Cardiologists

A cardiologist is a doctor who specializes in treating problems with the cardiovascular system, such as conditions of the heart and blood vessels.14 Increased incidence of cardiovascular disease, including myocardial infarction, cerebrovascular disease, and cardiac arrhythmias, are all highly associated with AAV. Accelerated atherosclerotic disease may develop as a direct result of the disease’s activity and endothelial damage, as a side effect of treatment, or for reasons that are presently unclear.15

Read more about AAV complications

Ophthalmologists 

An ophthalmologist is a medical professional who specializes in eye disorders and vision treatment.16 Patients with AAV can present with painful, red eyes (scleritis) and

diplopia with proptosis, which is caused by retro-orbital granulomatous masses.13,17

Otolaryngologists 

An otolaryngologist, also called an ENT doctor, is a specialized doctor trained in the diagnosis and treatment of diseases of the ear, nose, and throat.18 Patients with AAV often present with upper respiratory tract symptoms or signs, such as nasal obstruction and chronic recurrent infections. Nasal bloody discharge, nasal crusting and obstruction may also be observed.19

Dermatologists

A dermatologist is a doctor who specializes in diseases of the skin, hair, and nails.20

Lesions of skin are among the common, noticeable and easily detectable early AAV manifestations, allowing for immediate disease recognition and diagnosis based on the clinical pattern and histological findings. Petechiae/purpura have been the most frequently noticed clinical manifestations across all AAV variants.21

Dietitians and Nutritionists 

A dietitian is an expert in treating disease-related nutritional dysfunction. Patients with AAV often experience nutritional challenges that are both clinical manifestations of the disease and side effects of immunosuppressive treatments. Close monitoring of the nutritional status of patients can help avoid complications. Therefore, dietitians and nutritionists also play an important role in AAV care team.22

Read more about AAV diet and nutrition

References

  1. Yates M, Watts R. ANCA-associated vasculitis. Clin Med (Lond). 2017;17(1):60-64. doi:10.7861/clinmedicine.17-1-60
  2. Qasim A, Patel JB. ANCA positive vasculitis. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2022. Updated May 29, 2022. Accessed March 14, 2023. 
  3. Primary care. American Academy of Family Physicians (AAFP). Accessed March 14, 2023. 
  4. What is a rheumatologist? American College of Rheumatology. Updated May 2021. Accessed March 14, 2023. 
  5. Vasculitis. Rheumatology Advisor. Updated August 9, 2022. Accessed March 14, 2023.
  6. Paterick TE, Patel N, Tajik AJ, Chandrasekaran K. Improving health outcomes through patient education and partnerships with patients. Proc (Bayl Univ Med Cent). 2017;30(1):112-113. doi:10.1080/08998280.2017.11929552
  7. Huizen J. What is a nephrologist? Medical News Today. Updated October 16, 2019. Accessed March 14, 2023.
  8. Galesic K, Ljubanovic D, Horvatic I. Treatment of renal manifestations of ANCA-associated vasculitis. J Nephropathol. 2013;2(1):6-19. doi:10.5812/nephropathol.8971
  9. Aronson JK. What do clinical pharmacologists do? A questionnaire survey of senior UK clinical pharmacologists. Br J Clin Pharmacol. 2012;73(2):161-169. doi:10.1111/j.1365-2125.2011.04079.x
  10. Kimble B. Know your providers: what does a pulmonologist do? American Lung Association. Updated November 17, 2022. Accessed March 14, 2023.
  11. Homma S, Suzuki A, Sato K. Pulmonary involvement in ANCA-associated vasculitis from the view of the pulmonologist. Clin Exp Nephrol. 2013;17(5):667-671. doi:10.1007/s10157-012-0710-7
  12. What is a neurologist? University of Rochester Medical Center. Accessed March 19, 2023.
  13. Hunter RW, Welsh N, Farrah TE, Gallacher PJ, Dhaun N. ANCA associated vasculitis. BMJ. 2020;369:m1070. doi:10.1136/bmj.m1070
  14. Cardiologist. Cleveland Clinic. Accessed March 19, 2023.
  15. Floyd L, Morris AD, Woywodt A, Dhaygude A, Cardiovascular disease and ANCA-associated vasculitis: are we missing a beat? Clin Kid J. 2022;15(4):618–623. https://doi.org/10.1093/ckj/sfac009
  16. What is an ophthalmologist? American Academy of Ophthalmology. Accessed March 19, 2023.
  17. Weiner M, Segelmark M. The clinical presentation and therapy of diseases related to anti-neutrophil cytoplasmic antibodies (ANCA). Autoimmun Rev. 2016;15(10):978-982. doi:10.1016/j.autrev.2016.07.016
  18. Otolaryngologist. National Cancer Institute at the National Institutes of Health. Accessed March 19, 2023.
  19. Martinez Del Pero M, Sivasothy P. Vasculitis of the upper and lower airway. Best Pract Res Clin Rheumatol. 2009;23(3):403-417. doi:10.1016/j.berh.2009.02.003
  20. What is a dermatologist? American Academy of Dermatology Association. Accessed March 20, 2023.
  21. Micheletti RG, Chiesa Fuxench Z, Craven A, et al. Cutaneous manifestations of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 2020;72(10):1741-1747. doi:10.1002/art.41310
  22. Dellaripa PF, Howard D. Nutritional issues in vasculitis In: Coleman LA, ed. Nutrition and Rheumatic Disease. Humana Press;2008:215-226. Accessed March 20, 2023.

Reviewed by Hasan Avcu, MD, on 3/16/2023.

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