Difficulties in diagnosing ANCA-associated vasculitis in pulmonology practice: a clinical case of granulomatosis with polyangiitis with pulmonary destruction cavity

This article presents a clinical case of granulomatosis with polyangiitis (ANCA-associated vasculitis) in a 66-year-old patient, a long-term smoker, with an initial diagnosis of bilateral polysegmental pneumonia with lung tissue destruction. The lack of response to multicomponent antibacterial therapy, the appearance of extrapulmonary manifestations (hemorrhagic rash, arthralgia, hearing loss, glomerulonephritis), and the detection of high titers of antibodies to neutrophil cytoplasm (ANCA) and proteinase-3 allowed for verification of the diagnosis. The diagnosis was confirmed by a multidisciplinary consultation. Timely administration of glucocorticosteroids and cyclophosphamide resulted in clinical and laboratory remission. The need for alertness for systemic vasculitis in patients with protracted destructive pneumonia resistant to antibiotics is emphasized.

1. Байрашевская А.В., Дегтярова Н.Д., Раденска-Лоповок С.Г. АНЦА-ассоциированные васкулиты. Архив патологии. 2022;84(1):50‑58 DOI: 10.17116/patol20228401150

2. Kitching AR, Anders HJ, Basu N, Brouwer E, Gordon J, Jayne DR, et al. ANCA-associated vasculitis. Nat Rev Dis Primers. 2020;6(1):71. DOI: 10.1038/s41572-020-0204-y

3. Клименко А.А., Логинова Т.К., Саакян Ю.М., Петрикова В.И., Андрияшкина Д.Ю. Поражение органа слуха и верхних дыхательных путей при васкулитах, ассоциированных с антинейтрофильными цитоплазматическими антителами. Клиницист. 2024;18(2):12-20.

4. Zhao WM, Wang ZJ, Shi R, Zhu YY, Zhang S, Wang RF, et al. Environmental factors influencing the risk of ANCA-associated vasculitis. Front Immunol. 2022;13:991256. DOI: 10.3389/fimmu.2022.991256

5. Storrar J, Launiyomchon S, Amin Y, Raza S, Chinnadurai R, O’Riordan E, et al. Shifting Sands or ANCA-ed in Place? The Epidemiology of ANCA-Associated Vasculitis over 2 Decades at a Large Tertiary Centre. Glomerular Dis. 2025;6(1):10-25. DOI: 10.1159/000549121. PMID: 41439239; PMCID: PMC12721725.

6. Kronbichler A, Bajema IM, Bruchfeld A, Mastroianni Kirsztajn G, Stone JH. Diagnosis and management of ANCA-associated vasculitis. Lancet. 2024;403(10427):683-698. DOI: 10.1016/S0140-6736(23)01736-1

7. Rathmann J, Segelmark M, Mohammad AJ. Evaluation of the ACR/EULAR 2022 criteria for classification of ANCA-associated vasculitis in a population-based cohort from Sweden. Rheumatology (Oxford). 2024;63(7):1965-1972. DOI: 10.1093/rheumatology/kead516

8. Скворцов А.В., Литвинова М.А., Акулкина Л.А., Буланов Н.М., Новиков П.И., Моисеев С.В. Клинико-рентгенологические варианты поражения легких при АНЦА-ассоциированных васкулитах: ретроспективное исследование. Вестник Российской академии медицинских наук. 2024;79(4):338-345. DOI: 10.15690/vramn17982

9. Мазуров В.И., Беляева И.Б., Чудинов А.Л., Инамова О.В. Осложнения и причины летальности у пациентов с первичными некротизирующими системными васкулитами. Медицинский алфавит. 2021;(33):30-34 DOI: 10.33667/2078-5631-2021-33-30-34

10. Hellmich B, Sanchez-Alamo B, Schirmer JH, Berti A, Blockmans D, Cid MC, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis. 2024;83(1):30-47. DOI: 10.1136/ard-2022-223764

11. Robson JC, Grayson PC, Ponte C, Suppiah R, Craven A, Judge A,et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Granulomatosis With Polyangiitis. Arthritis Rheumatol. 2022;74(3):393-399. DOI: 10.1002/art.41986

12. Rodrigues F, Oliveira Sá AI, Mendes M, Macedo E, Apolinário I. Granulomatosis With Polyangiitis: A Clinical Case. Cureus. 2022;14(12):e32410. DOI: 10.7759/cureus.32410

13. Bandari V, Apenteng S, Kaul A. A Rare Case of Limited Granulomatosis with Polyangiitis Presenting as Bilateral Parotitis. Eur J Case Rep Intern Med. 2024;11(12):004992. DOI: 10.12890/2024_004992

14. Gormley A, Green P. Granulomatosis with polyangiitis mimicking multisystem pyoderma gangrenosum: A case report. SAGE Open Med Case Rep. 2024;12:2050313X241304229. DOI: 10.1177/2050313X241304229

15. Hernández IA, Friesen R, McGaw T, Levin L. Granulomatosis with polyangiitis mistaken as a temporomandibular joint disorder: A case report. Clin Adv Periodontics. 2023;13(2):106- 109. DOI: 10.1002/cap.10189

16. Liu J, Zhang B. Bilateral corneoscleritis with retrocorneal plaque as the initial presentation of granulomatosis with polyangiitis. BMC Ophthalmol. 2025;25(1):187. DOI: 10.1186/s12886-025-04031-w