Lung Involvement Patterns in ANCA-Associated Vasculitis: a Retrospective Study

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Abstract

Background. Pulmonary involvement in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is an important cause of mortality. The main variants of AAV are granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA).

Aims — to define the prevalence of pulmonary involvement in AAV patients and describe its computed tomography (CT)-patterns.

Methods. A retrospective study was conducted. The study included patients with GPA and MPA. The diagnosis was established in accordance with the American College of Rheumatology (1990) criteria of GPA or the Chapel-Hill (2012) conference AAV definitions, as well as the classification criteria of GPA and MPA of the American College of Rheumatology and the European Alliance of Associations for Rheumatology (2022). All patients underwent chest CT. The primary endpoint was lung involvement, as determined by the results of a clinical and instrumental examination.

Results. 243 patients (median age of onset 49.0 (35.0; 59.0); 167 (68.7%) women) with AAV were included in the study, including 171 with GPA and 72 with MPA. Lung damage was established in 175 (72.0%) patients (121 (70.8%) in GPA and 54 (75.0%) in MPA). The following variants of pulmonary involvement in AAV were registered: parenchymal infiltrates — 103 (58.9%); alveolar hemorrhage — 33 (18.9%); nodular disease — 58 (33.1%); cavities — 28 (16.0%); subclinical interstitial lung disease (ILD) — 43 (24.6%); ILD — 8 (4.6%); signs of fibrosis — 135 (77.1%); bronchiectasis — 24 (13.7%); and pleural effusion — 3 (1.7%). In GPA patients, cavities were more prevalent (p < 0.001), so alveolar hemorrhage was in MPA (p < 0.001). PR3-ANCA (OR: 3.25; 95% CI: 1.40–7.49) and general symptoms (fever, weight loss) (OR: 2.41; 95% CI: 1.28–4.54) were associated with pulmonary damage according to the logistic regression model.

Conclusions. Pulmonary involvement in AAV is frequent. Among 243 patients with MPA and GPA who underwent a CT scan of the chest, 72% had lung abnormalities. These findings may have important implications for the diagnosis and management of AAV lung manifestations.

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About the authors

Alexey V. Skvortsov

I.M. Sechenov First Moscow State Medical University (Sechenov University)

Author for correspondence.
Email: alex.v.skvortsov13@yandex.ru
ORCID iD: 0000-0001-8743-5207
SPIN-code: 7955-0655

Assistant Lecturer, PhD Student

Russian Federation, Moscow

Maria A. Litvinova

I.M. Sechenov First Moscow State Medical University (Sechenov University)

Email: maria.litvinova.2015@yandex.ru
ORCID iD: 0000-0002-3136-2755
SPIN-code: 7722-2462

MD, Assistant Lecturer, PhD Student

Russian Federation, Moscow

Larisa A. Akulkina

I.M. Sechenov First Moscow State Medical University (Sechenov University)

Email: akullar.ru@mail.ru
ORCID iD: 0000-0002-4307-8882

MD, PhD, Head of the Department of Occupational Medicine and Pulmonology

Russian Federation, Moscow

Nikolay M. Bulanov

I.M. Sechenov First Moscow State Medical University (Sechenov University)

Email: nmbulanov@gmail.com
ORCID iD: 0000-0002-3989-2590
SPIN-code: 7408-5706

MD, PhD, Associate Professor

Russian Federation, Moscow

Pavel I. Novikov

I.M. Sechenov First Moscow State Medical University (Sechenov University)

Email: novikov-pavel@mail.ru
ORCID iD: 0000-0003-0148-5655
SPIN-code: 9876-3122

MD, PhD, Head of the Rheumatology Department

Russian Federation, Moscow

Sergey V. Moiseev

I.M. Sechenov First Moscow State Medical University (Sechenov University)

Email: avt420034@gmail.com
ORCID iD: 0000-0002-7232-4640
SPIN-code: 3462-7884

MD, PhD, Professor

Russian Federation, Moscow

References

  1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1–11. doi: https://doi.org/10.1002/art.37715
  2. Moiseev S, Cohen Tervaert JW, Arimura Y, et al. 2020 international consensus on ANCA testing beyond systemic vasculitis. Autoimmun Rev. 2020;19(9):102618. doi: https://doi.org/10.1016/j.autrev.2020.102618
  3. Lane SE, Watts RA, Shepstone L, et al. Primary systemic vasculitis: clinical features and mortality. QJM. 2005;98(2):97–111. doi: https://doi.org/10.1093/qjmed/hci015
  4. Sánchez Álamo B, Moi L, Bajema I, et al. Long-term outcomes and prognostic factors for survival of patients with ANCA-associated vasculitis. Nephrol Dial Transplant. 2023;38(7):1655–1665. doi: https://doi.org/10.1093/ndt/gfac320
  5. Walsh M, Merkel PA, Peh CA, et al. Plasma Exchange and Glucocorticoids in Severe ANCA-Associated Vasculitis. N Engl J Med. 2020;382(7):622–631. doi: https://doi.org/10.1056/NEJMoa1803537
  6. Guillevin L, Pagnoux C, Karras A, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med. 2014;371(19):1771–1780. doi: https://doi.org/10.1056/NEJMoa1404231
  7. Solans-Laqué R, Fraile G, Rodriguez-Carballeira M, et al. Clinical characteristics and outcome of Spanish patients with ANCA-associated vasculitides: Impact of the vasculitis type, ANCA specificity, and treatment on mortality and morbidity. Medicine (Baltimor). 2017;96(8):e6083. doi: https://doi.org/10.1097/MD.0000000000006083
  8. Zhou P, Li Z, Gao L, et al. Pulmonary involvement of ANCA-associated vasculitis in adult Chinese patients. BMC Pulm Med. 2022;22(1):35. doi: https://doi.org/10.1186/s12890-022-01829-y
  9. Nada AK, Torres VE, Ryu JH, et al. Pulmonary fibrosis as an unusual clinical manifestation of a pulmonary-renal vasculitis in elderly patients. Mayo Clin Proc. 1990;65(6):847–856. doi: https://doi.org/10.1016/s0025-6196(12)62575-0
  10. Arimura Y, Minoshima S, Tanaka U, et al. Pulmonary involvement in patients with myeloperoxidase specific-antineutrophil cytoplasmic antibody. Ryumachi. 1995;35(1):46–55.
  11. Zhou P, Li Z, Gao L, et al. Patterns of Interstitial Lung Disease and Prognosis in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Respiration. 2023;102(4):257–273. doi: https://doi.org/10.1159/000529085
  12. Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum. 1990;33(8):1101–1107. doi: https://doi.org/10.1002/art.1780330807
  13. Robson JC, Grayson PC, Ponte C, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis. Ann Rheum Dis. 2022;81(3):315–320. doi: https://doi.org/10.1136/annrheumdis-2021-221795
  14. Suppiah R, Robson JC, Grayson PC, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis. Ann Rheum Dis. 2022;81(3):321–326. doi: https://doi.org/10.1136/annrheumdis-2021-221796
  15. Travis WD, Costabel U, Hansell DM, et al. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188(6):733–748. doi: https://doi.org/10.1164/rccm.201308-1483ST
  16. Бекетова Т.В. Гранулематоз с полиангиитом, патогенетически связанный с антинейтрофильными цитоплазматическими антителами: особенности клинического течения // Научно-практическая ревматология. — 2012. — Т. 50. — № 6. — С. 19–28. [Beketova TV. Granulomatosis with polyangiitis, which is pathogenetically associated with antineutrophil cytoplasmic antibodies: clinical features. Rheumatology Science and Practice. 2012;50(6):19–28. (In Russ).]
  17. Бекетова Т.В. Микроскопический полиангиит, ассоциированный с антинейтрофильными цитоплазматическими антителами: особенности клинического течения // Teрапевтический архив. — 2015. — Т. 87. — № 5. — С. 33–46. [Beketova TV. Microscopic polyangiitis associated with antineutrophil cytoplasmic antibodies: Clinical features. Therapeutic Archive. 2015;87(5):33–46. (In Russ).] doi: https://doi.org/10.17116/terarkh201587533-46
  18. Захарова Е.В., Яковлев В.Н., Виноградова О.В., и др. ANCA-ассоциированные васкулиты с поражением легких и почек: клинико-морфологическая характеристика, лечение, исходы // Клиническая медицина. — 2013. — Т. 91. — № 7. — С. 38–42. [Zakharova EV, Iakovlev VN, Vinogradova OV, et al. ANCA-associated vasculitis affecting lungs and kidneys: clinical and morphological characteristic, treatment, outcomes. Klin Med (Mosk). 2013;91(7):38–43. (In Russ).]
  19. Щеголева Е.М., Буланов Н.М., Виноградова Е.С., и др. Варианты течения и исходы микроскопического полиангиита // Клиническая фармакология и терапия. — 2018. — Т. 23. — № 3. — С. 35–40. [Shchegoleva EM, Bulanov NM, Vinogradova ES, et al. Clinical variants and outcomes of microscopic polyangiitis. Clinical pharmacology and therapy. 2018;23(3):35–40. (In Russ).]
  20. Mohammad AJ, Mortensen KH, Babar J, et al. Pulmonary Involvement in Antineutrophil Cytoplasmic Antibodies (ANCA)-associated Vasculitis: The Influence of ANCA Subtype. J Rheumatol. 2017;44(10):1458–1467. doi: https://doi.org/10.3899/jrheum.161224
  21. Kloth C, Thaiss WM, Beck R, et al. Potential role of CT-textural features for differentiation between viral interstitial pneumonias, pneumocystis jirovecii pneumonia and diffuse alveolar hemorrhage in early stages of disease: a proof of principle. BMC Med Imaging. 2019;19(1):39. doi: https://doi.org/10.1186/s12880-019-0338-0
  22. Kambhatla S, Vipparthy S, Manadan AM. Rheumatic diseases associated with alveolar hemorrhage: analysis of the national inpatient sample. Clin Rheumatol. 2023;42(4):1177–1183. doi: https://doi.org/10.1007/s10067-022-06449-9
  23. Bhushan A, Choi D, Maresh G, et al. Risk factors and outcomes of immune and non-immune causes of diffuse alveolar hemorrhage: a tertiary-care academic single-center experience. Rheumatol Int. 2022;42(3):485–492. doi: https://doi.org/10.1007/s00296-021-04842-2
  24. Unizony S, Villarreal M, Miloslavsky EM, et al. Clinical outcomes of treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis based on ANCA type. Ann Rheum Dis. 2016;75(6):1166–1169. doi: https://doi.org/10.1136/annrheumdis-2015-208073
  25. West S, Arulkumaran N, Ind PW, et al. Diffuse Alveolar Haemorrhage in ANCA-associated Vasculitis. Intern Med. 2013;52(1):5–13. doi: https://doi.org/10.2169/internalmedicine.52.8863
  26. Bahmer T, Romagnoli M, Girelli F, et al. The use of auto-antibody testing in the evaluation of interstitial lung disease (ILD) — A practical approach for the pulmonologist. Respir Med. 2016;113:80–92. doi: https://doi.org/10.1016/J.RMED.2016.01.019
  27. Borie R, Crestani B. Antineutrophil Cytoplasmic Antibody-Associated Lung Fibrosis. Semin Respir Crit Care Med. 2018;39(4):465–470. doi: https://doi.org/10.1055/s-0038-1669914
  28. Sebastiani M, Luppi F, Sambataro G, et al. Interstitial Lung Disease and Anti-Myeloperoxidase Antibodies: Not a Simple Association. J Clin Med. 2021;10(12):2548. doi: https://doi.org/10.3390/JCM10122548
  29. Alba MA, Flores-Suárez LF, Henderson AG, et al. Interstital lung disease in ANCA vasculitis. Autoimmuny Rev. 2017;16(7):722–729. doi: https://doi.org/10.1016/J.AUTREV.2017.05.008
  30. Sebastiani M, Manfredi A, Vacchi C, et al. Epidemiology and management of interstitial lung disease in ANCA-associated vasculitis. Clin Exp Rheumatol. 2020;38Suppl 124(2):221–231.
  31. Liu GY, Ventura IB, Achtar-Zadeh N, et al. Prevalence and Clinical Significance of Antineutrophil Cytoplasmic Antibodies in North American Patients with Idiopathic Pulmonary Fibrosis. Chest. 2019;156(4):715–723. doi: https://doi.org/10.1016/j.chest.2019.05.014

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