How can combined ENT vasculitis clinics improve diagnosis, treatment and ongoing care for patients with AAV? This article outlines a multidisciplinary approach.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of rare autoimmune conditions characterised by inflammation of small- to medium-sized blood vessels. They include granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis), microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) [1].
Although rare, with an estimated prevalence of ~200 per million, AAV carries a disproportionate burden of morbidity due to multisystem involvement, a relapsing disease course and progressive organ damage.
The team assessing a patient with subglottic stenosis during the combined airway vasculitis clinic at Charing Cross Hospital: Stephen McAdoo, Chad Al Yagchi (consultant laryngologist), Laura Gosselin (airway research fellow), Yamin Kassir (renal research fellow).
AAV is particularly relevant to ENT clinicians. Most patients with GPA, and many with EGPA, develop ear, nose and throat involvement, and despite its rarity, the average UK ENT consultant can expect to see a new case of GPA or EGPA every two years [2]. ENT features are often the earliest sign of disease. Patients may present with chronic rhinosinusitis, with or without polyps, nasal perforations and saddling, otitis media with effusion, sensorineural hearing loss and subglottic disease [3]. However, these symptoms are frequently referred and managed as isolated ENT problems, risking delayed recognition of an underlying systemic condition.
This separation between local and systemic care is a key challenge in AAV. ENT manifestations are not simply ‘local’ or ‘limited’ disease; they are part of a broader inflammatory illness. Sinonasal symptoms may reflect active disease and can precede systemic relapse, but they may also arise from established structural damage or secondary infection. Airway involvement, such as subglottic stenosis, can progress independently of systemic disease and does not always correlate with systemic markers. Effective treatment of ENT manifestations often requires a combination of systemic immunosuppression and targeted local or surgical therapies, guided by an assessment of overall disease activity as well as patient-specific factors such as treatment risk. All this complexity requires a multidisciplinary approach.
"ENT manifestations are not simply ‘local’ or ‘limited’ disease; they are part of a broader inflammatory illness"
Recent guidance from the British Society for Rheumatology (BSR) highlights the need for more coordinated care [4]. The 2025 BSR recommendations for AAV go beyond treatment algorithms and include, for the first time, clear guidance on service delivery. They emphasise the importance of multidisciplinary working, structured care pathways and access to specialist expertise. ENT involvement, including sinonasal and airway disease, is recognised as a key component of disease assessment, but the broader message is that AAV should be managed within integrated services rather than across isolated specialties.
This aligns closely with findings from the VOICES study, led by Rosemary Hollick, which explored patient experience in vasculitis [5]. A consistent theme from VOICES was the burden of fragmented care. Patients described difficulties navigating multiple appointments, inconsistent communication between specialties and uncertainty about who was coordinating their treatment. Importantly, patients identified joined-up care and access to clinicians with vasculitis expertise as key priorities.
Combined medical vasculitis-ENT clinics offer a practical way of addressing these issues. This model has been implemented in several UK centres, including our own, where a rhinologist joins the vasculitis clinic every fortnight to assess patients, perform nasolaryngoendoscopy and contribute to real-time management decisions. In parallel, vasculitis physicians attend dedicated airway clinics, supporting the assessment of patients with subglottic disease alongside laryngology colleagues in a coordinated setting. This approach reduces fragmentation and allows systemic and local disease to be evaluated together, supporting a more coherent approach to care. At our centre over five years, 171 vasculitis patients were seen in the combined ENT vasculitis service. This concentration of patients fosters the development of disease-specific expertise that would not otherwise be possible.
One of the main benefits is improved diagnostic accuracy. Patients presenting with predominantly ENT symptoms may otherwise experience delays before vasculitis is recognised and treated. Joint assessment helps identify relevant clinical patterns earlier and supports more timely investigation and treatment.
Combined clinics also improve recognition of relapse. ENT symptoms are often the earliest indicator of disease activity, but they can be difficult to interpret in isolation. A shared clinical review makes it easier to determine whether symptoms represent active inflammation requiring escalation of immunosuppression, or established damage that is better managed with local or surgical approaches.
Decision-making is also more consistent. Management of AAV often involves balancing medical therapy with procedural intervention. For example, patients with persistent sinonasal disease or subglottic stenosis may require surgery, but outcomes depend on adequate control of underlying inflammation. Operating during active disease increases the risk of complications and recurrence. A combined clinic allows these decisions to be made collaboratively, in line with guideline recommendations.
Alongside their clinical role, these clinics provide a valuable setting for training and education, allowing resident doctors to develop experience in recognising vasculitis-related ENT disease and in multidisciplinary decision-making. They also provide a platform for audit and research, such as the development of improved clinical tools, imaging approaches or biomarkers to assess ENT disease activity. In addition, they enable prospective evaluation of care models, helping to define their impact on clinical outcomes and patient experience.
There is emerging evidence to support this model. Experience from UK centres suggests that combined ENT vasculitis clinics can improve diagnostic confidence, reduce delays in treatment and support more coordinated care [6,7]. Although formal outcome data for combined clinics are still limited, findings from the VOICES study suggest that patients with vasculitis managed within specialist multidisciplinary services experience better outcomes, including fewer infections, fewer emergency admissions and improved survival. Taken together, these data support the value of integrated, specialist care models in vasculitis.
"Patients described difficulties navigating multiple appointments, inconsistent communication between specialties and uncertainty about who was coordinating their treatment"
Despite these advantages, implementation can be challenging. Coordinating clinicians across specialties requires time and organisational support, and funding models are not always aligned with multidisciplinary care. Access to vasculitis expertise may also vary between centres. However, the direction of travel is clear. Both national guidance and patient-reported experience highlight the need for better integration of care, and it is hoped that the BSR recommendations can be used as a practical tool to inform commissioning decisions and enable service leads to develop and sustain these models.
In summary, ENT involvement is a central feature of AAV and plays an important role in both diagnosis and ongoing monitoring. Managing these patients within separate specialty pathways is increasingly difficult to justify. Combined medical vasculitis-ENT clinics offer a practical and guideline-supported approach that addresses both clinical complexity and patient priorities. As services continue to evolve, this model is likely to become an essential part of routine vasculitis care.
References
1. Kitching AR, Anders HJ, Basu N, et al. ANCA-associated vasculitis. Nat Rev Dis Primers 2020;6(1):71.
2. Coates ML, Martinez Del Pero M. Updates in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis for the ENT surgeon. Clin Otolaryngol 2020;45(3):316–26.
3. Wasserman I, Chari DA, Gray ST, et al. Ear, Nose, and Throat Manifestations of Vasculitis and Other Systemic Autoimmune Diseases: Otologic, Sinus, and Airway. Rheum Dis Clin North Am 2023;49(3):633–45.
4. Biddle K, Jade J, Wilson-Morkeh H, et al. The 2025 British Society for Rheumatology management recommendations for ANCA-associated vasculitis. Rheumatology (Oxford) 2025;64(8):4470–94.
5. Hollick RJ, James WRG, Nicoll A, et al. Identifying key health system components associated with improved outcomes to inform the re-configuration of services for adults with rare autoimmune rheumatic diseases: a mixed-methods study. Lancet Rheumatol 2024;6(6):e361–73.
6. O’Malley L, Chanouzas D, Logan S, et al. 292. UTILITY OF A ONE-STOP JOINT ENT-VASCULITIS CLINIC IN THE MANAGEMENT OF RELAPSING ANCA-ASSOCIATED VASCULITIS. Rheumatology 2019;58(Supplement_2).
7. Stavrakas M, Smith R, Akil M, et al. Added value of Joint ENT-Rheumatology clinic in the management of ANCA-associated vasculitis: One year’s experience. Am J Otolaryngol 2022;43(4):103485.
Declaration of competing interests: None declared.
