ANCA-associated vasculitis (AAV) is a complex autoimmune condition that causes inflammation in small and medium-sized blood vessels, potentially affecting multiple organs. Effective management requires a coordinated effort from various medical specialists, not just one. This article outlines the key roles within a typical AAV care team and how patients can best navigate this multidisciplinary approach.
Why Coordination Matters
AAV can manifest differently in each patient, impacting kidneys, lungs, ears, nerves, or skin. The body’s blood vessels become inflamed, cutting off oxygen and nutrients to vital organs. Left unchecked, this can lead to organ damage and even life-threatening complications. Because the disease is systemic, a single doctor can’t address all potential issues. A team ensures comprehensive, timely care.
The Core Specialists
Rheumatologist: The Team Leader
In most cases, a rheumatologist takes the lead in managing AAV. This specialist diagnoses and treats autoimmune conditions affecting joints, muscles, and the immune system. They will initiate treatment with immunosuppressants like steroids and biologics (such as rituximab) and monitor long-term remission.
Nephrologist: Protecting Kidney Function
Kidney involvement occurs in 70–90% of AAV cases. A nephrologist monitors kidney health closely, even with mild inflammation, to prevent long-term damage. Their role includes diagnosis, treatment, and education about the disease’s impact on renal function.
Pulmonologist: Addressing Lung Complications
Lung issues affect up to 80% of AAV patients. A pulmonologist identifies and manages lung scarring, bleeding, and shortness of breath. Early detection is critical, as lung involvement can be severe.
Otolaryngologist (ENT): Treating Upper Respiratory Symptoms
Many AAV cases begin with symptoms that mimic colds or upper respiratory infections. An ENT specialist distinguishes between these and AAV-related inflammation in the ears, nose, and throat, preventing irreversible organ damage. They also address saddle nose deformity, a characteristic AAV complication.
Expanding the Team When Needed
If AAV affects other areas, additional specialists are brought in:
- Neurologist: For nerve inflammation causing weakness, tingling, or difficulty walking (common in certain AAV types).
- Ophthalmologist: For eye inflammation that threatens vision. About half of granulomatosis with polyangiitis (GPA) cases involve eye problems.
- Dermatologist: For skin rashes, lesions, or discoloration indicative of deeper AAV activity.
- Vascular Surgeon: In rare but severe cases, to repair blood vessels damaged by inflammation. Surgery is reserved for limb- or organ-threatening complications.
Empowering Patients to Coordinate Care
Patients often need to be their own advocates, ensuring seamless communication between specialists. Here’s how:
- Maintain an updated medication list: Include dosages, start dates, and prescribing doctors.
- Identify a point person: Know who to contact for symptom flare-ups.
- Use shared patient portals: Access clinic notes and lab results to stay organized.
- Keep your rheumatologist informed: After visits with other specialists, update them on any changes.
- Request a written treatment plan: Include medication schedules, lab monitoring, and red-flag symptoms.
Seeking care at specialized vasculitis clinics, where multidisciplinary teams already work together, is recommended.
The Bottom Line
ANCA-associated vasculitis requires a team-based approach to ensure comprehensive care. By understanding the roles of each specialist and actively coordinating their own treatment, patients can improve outcomes and minimize long-term complications.
Sources
Rheumatologist. American College of Rheumatology.
ANCA-Associated Vasculitis. Cleveland Clinic. October 15, 2025.
Huang X et al. Antineutrophil Cytoplasmic Antibody-Associated Vasculitis With Acute Kidney Injury: Short-Term Recovery Predicts Long-Term Outcome. Frontiers in Immunology. July 9, 2021.
Sanchez-Alamo B et al. Long-Term Outcome of Kidney Function in Patients with ANCA-Associated Vasculitis. Nephrology Dialysis Transplantation. September 2024.
Zhou P et al. Pulmonary Involvement of ANCA-Associated Vasculitis in Adult Chinese Patients. BMC Pulmonary Medicine. January 12, 2022.
Coates ML et al. Ear, Nose, Throat and Airways Disease in Granulomatosis With Polyangiitis. Rheumatology (Oxford, England). June 16, 2021.
Padoan R et al. Ear, Nose, and Throat in Anca-Associated Vasculitis: A Comprehensive Review. Vessel Plus. May 23, 2021.
Granulomatosis With Polyangiitis. Johns Hopkins Medicine.
Villa A. Peripheral Neuropathy as Initial Manifestation of Churg Strauss Syndrome. International Archives of Internal Medicine. 2019.
Koike H et al. ANCA-Associated Vasculitic Neuropathies: A Review. Neurology and Therapy. January 19, 2022.
Byszewska J et al. Ocular Involvement of Granulomatosis with Polyangiitis. Journal of Clinical Medicine. July 2, 2023.
Shakshouk H et al. Cutaneous Manifestations of ANCA-Associated Vasculitis: A Retrospective Review of 211 Cases With Emphasis on Clinicopathologic Correlation and ANCA Status . International Journal of Dermatology. February 2023.
Hollick R. Specialist Nurses in Vasculitis Care: Evidence and a Path Forward. Vasculitis International.
FDA Pharmacists Help You Use Medicines Safely. U.S. Food and Drug Administration. August 31, 2023.
