Difficulties in diagnosing eosinophilic granulomatosis with polyangiitis in clinical practice
DOI: 10.32364/2587-6821-2024-8-3-8
N.A. Karoli, T.V. Kanaeva, N.M. Nikitina
V.I. Razumovskiy Saratov State Medical University, Saratov, Russian Federation
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem immune-mediated vasculitis with damage to medium and small vessels (including capillaries, venules and veins), first described in 1951. The etiology and pathogenesis of this disease associated with a combination of immunocomplex and allergic mechanisms have not been fully studied, and there are no generally accepted techniques to initial therapy. The diversity of the clinical picture at the disease onset, the gradual manifestation of signs, the need to take into account not only clinical but also laboratory parameters, makes it difficult to make a correct diagnosis in the early stages. This article describes a clinical case of a relatively late diagnosis of EGPA, which may be due to a lack of practitioner awareness about such a rare disease and a late referral to a rheumatologist. Not only morphological verification and immunological tests of patients can speed up diagnosis, but also discussion of such clinical cases in the medical community. Currently, the EGPA therapy possibilities have been expanded by including the disease in indications for biological treatments aimed at interleukin 5, which will improve the disease prognosis and reduce the burden of long-term hormonal therapy.
Keywords: eosinophilic granulomatosis with polyangiitis, vasculitis, eosinophilia, differential diagnosis, bronchial asthma, clinical case.
For citation: Karoli N.A., Kanaeva T.V., Nikitina N.M. Difficulties in diagnosing eosinophilic granulomatosis with polyangiitis in clinical practice. Russian Medical Inquiry. 2024;8(3):171–175 (in Russ.). DOI: 10.32364/2587-6821-2024-8-3-8.
Nina A. Karoli — Dr. Sc. (Med.), Professor of the Department of Hospital Therapy of the Faculty of Medicine, V.I. Razumovskiy Saratov State Medical University; 112, Bolshaya Kazachya str., Saratov, 410012, Russian Federation; ORCID iD 0000-0002-7464-826X.
Tatyana V. Kanaeva — Assistant of the Department of Hospital Therapy of the Faculty of Medicine, V.I. Razumovskiy Saratov State Medical University; 112, Bolshaya Kazachya str., Saratov, 410012, Russian Federation; ORCID iD 0000-0002-9451-9318.
Natalia M. Nikitina — Dr. Sc. (Med.), Professor of the Department of Hospital Therapy of the Faculty of Medicine, V.I. Razumovskiy Saratov State Medical University; 112, Bolshaya Kazachya str., Saratov, 410012, Russian Federation; ORCID iD 0000-0002-0313-1191.
Contact information: Nina A. Karoli, e-mail: nina.karoli.73@gmail.com.
Financial Disclosure: no authors have a financial or property interest in any material or method mentioned.
There is no conflict of interest.
Received 23.01.2024.
Revised 15.02.2024.
Accepted 14.03.2024.
References
1. Trivioli G., Terrier B., Vaglio A. Eosinophilic granulomatosis with polyangiitis: understanding the disease and its management. Rheumatology (Oxford). 2020;59(Suppl 3):iii84–iii94. DOI: 10.1093/rheumatology/kez570.
2. Churg J., Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol. 1951;27(2):277–301. PMID: 14819261.
3. Jennette J.C., Falk R.J., Andrassy K. et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994;37(2):187–192. DOI: 10.1002/art.1780370206.
4. Jennette J.C., Falk R.J., Bacon P.A. et al. 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1–11. DOI: 10.1002/art.37715.
5. Grayson P.C., Ponte C., Suppiah R. et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis. Ann Rheum Dis. 2022;81(3):309–314. DOI: 10.1136/annrheumdis-2021-221794.
6. Harrold L.R., Patterson M.K., Andrade S.E. et al. Asthma drug use and the development of Churg-Strauss syndrome (CSS). Pharmacoepidemiol Drug Saf. 2007;16(6):620–626. DOI: 10.1002/pds.1353.
7. Gioffredi A., Maritati F., Oliva E., Buzio C. Eosinophilic granulomatosis with polyangiitis: an overview. Front Immunol. 2014;5:549. DOI: 10.3389/fimmu.2014.00549.
8. White J., Dubey S. Eosinophilic granulomatosis with polyangiitis: A review. Autoimmun Rev. 2023;22(1):103219. DOI: 10.1016/j.autrev.2022.103219.
9. Cottin V., Bel E., Bottero P. et al. Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Autoimmun Rev. 2017;16(1):1–9. DOI: 10.1016/j.autrev.2016.09.018.
10. Papo M., Sinico R.A., Teixeira V. et al. Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Rheumatology (Oxford). 2021;60(9):4355–4360. DOI: 10.1093/rheumatology/keaa805.
11. Liu S., Han L., Liu Y. et al. Clinical Significance of MPO-ANCA in Eosinophilic Granulomatosis With Polyangiitis: Experience From a Longitudinal Chinese Cohort. Front Immunol. 2022;13:885198. DOI: 10.3389/fimmu.2022.885198.
12. Chung S.A., Langford C.A., Maz M. et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Arthritis Rheumatol. 2021;73(8):1366–1383. DOI: 10.1002/art.41773.
13. Villa-Forte A. Eosinophilic granulomatosis with polyangiitis. Postgrad Med. 2023;135(sup1):52–60. DOI: 10.1080/00325481.2022.2134624.
14. Guillevin L., Lhote F., Gayraud M. et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore). 1996;75(1):17–28. DOI: 10.1097/00005792-199601000-00003.
15. Guillevin L., Pagnoux C., Seror R. et al. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 2011;90(1):19–27. DOI: 10.1097/MD.0b013e318205a4c6.
16. Emmi G., Bettiol A., Gelain E. et al. Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis. Nat Rev Rheumatol. 2023;19(6):378–393. DOI: 10.1038/s41584-023-00958-w.
17. Войцеховский В.В., Погребная М.В., Гоборов Н.Д. и др. Особенности диагностики и лечения синдрома Черджа—Стросс. Бюллетень физиологии и патологии дыхания. 2017;64:79–87. DOI: 10.12737/article_59361a30e7f1e7.92279749. Voytsekhovskiy V.V., Pogrebnaya M.V., Goborov N.D. et al. Peculiarities of diagnosis and treatment of Churg-Strauss syndrome. Bulletin Physiology and Pathology of Respiration. 2017;64:79–87 (in Russ.). DOI: 10.12737/article_59361a30e7f1e7.92279749.
18. Баранов А.А. Системные васкулиты: современные стандарты диагностики и лечения. РМЖ. 2005;24:1577–1581. Baranov A.A. Systemic vasculitis: modern standards of diagnosis and treatment. RMJ. 2005;24:1577–1581 (in Russ.).
19. Бекетова Т.В. Европейские (EULAR/ERA-EDTA) рекомендации по диагностике и лечению АНЦА-ассоциированных системных васкулитов. Научно-практическая ревматология. 2017;55(1):12–16. DOI: 10.14412/1995-4484-2017-12-16. Beketova T.V. The 2016 European (EULAR/ERA-EDTA) recommendations for the diagnosis and management of ANCA-associated systemic vasculitis. Rheumatology Science and Practice. 2017;55(1):12–16 (in Russ.). DOI: 10.14412/1995-4484-2017-12-16.
20. Wilhelm A.B., Pinsky S., Ahmad S. et al. Endomyocardial biopsy facilitates diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA): a case report. Cardiovasc Pathol. 2022;58:107407. DOI: 10.1016/j.carpath.2022.107407.
21. Fijolek J., Radzikowska E. Eosinophilic granulomatosis with polyangiitis — Advances in pathogenesis, diagnosis, and treatment. Front Med (Lausanne). 2023;10:1145257. DOI: 10.3389/fmed.2023.1145257.
22. Mukhtyar C., Lee R., Brown D. et al. Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Ann Rheum Dis. 2009;68(12):1827–1832. DOI: 10.1136/ard.2008.101279.
23. Groh M., Pagnoux C., Baldini C. et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Intern Med. 2015;26(7):545–553. DOI: 10.1016/j.ejim.2015.04.022.
24. Puéchal X., Pagnoux C., Baron G. et al. Adding Azathioprine to Remission-Induction Glucocorticoids for Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss), Microscopic Polyangiitis, or Polyarteritis Nodosa Without Poor Prognosis Factors: A Randomized, Controlled Trial. Arthritis Rheumatol. 2017;69(11):2175–2186. DOI: 10.1002/art.40205.
25. Steinfeld J., Bradford E.S., Brown J. et al. Evaluation of clinical benefit from treatment with mepolizumab for patients with eosinophilic granulomatosis with polyangiitis. J Allergy Clin Immunol. 2019;143(6):2170–2177. DOI: 10.1016/j.jaci.2018.11.041.
26. Pavord I.D., Korn S., Howarth P. et al. Mepolizumab for severe eosinophilic asthma (DREAM): a multicentre, double-blind, placebo-controlled trial. Lancet. 2012;380(9842):651–659. DOI: 10.1016/S0140-6736(12)60988-X.
27. Bel E.H., Wenzel S.E., Thompson P.J. et al. Oral glucocorticoid-sparing effect of mepolizumab in eosinophilic asthma. N Engl J Med. 2014;371(13):1189–1197. DOI: 10.1056/NEJMoa1403291.
28. Bettiol A., Urban M.L., Dagna L. et al. Mepolizumab for Eosinophilic Granulomatosis With Polyangiitis: A European Multicenter Observational Study. Arthritis Rheumatol. 2022;74(2):295–306. DOI: 10.1002/art.41943.
29. Wechsler M.E., Akuthota P., Jayne D. et al. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med. 2017;376(20):1921–1932. DOI: 10.1056/NEJMoa1702079.
2. Churg J., Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol. 1951;27(2):277–301. PMID: 14819261.
3. Jennette J.C., Falk R.J., Andrassy K. et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994;37(2):187–192. DOI: 10.1002/art.1780370206.
4. Jennette J.C., Falk R.J., Bacon P.A. et al. 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1–11. DOI: 10.1002/art.37715.
5. Grayson P.C., Ponte C., Suppiah R. et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis. Ann Rheum Dis. 2022;81(3):309–314. DOI: 10.1136/annrheumdis-2021-221794.
6. Harrold L.R., Patterson M.K., Andrade S.E. et al. Asthma drug use and the development of Churg-Strauss syndrome (CSS). Pharmacoepidemiol Drug Saf. 2007;16(6):620–626. DOI: 10.1002/pds.1353.
7. Gioffredi A., Maritati F., Oliva E., Buzio C. Eosinophilic granulomatosis with polyangiitis: an overview. Front Immunol. 2014;5:549. DOI: 10.3389/fimmu.2014.00549.
8. White J., Dubey S. Eosinophilic granulomatosis with polyangiitis: A review. Autoimmun Rev. 2023;22(1):103219. DOI: 10.1016/j.autrev.2022.103219.
9. Cottin V., Bel E., Bottero P. et al. Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Autoimmun Rev. 2017;16(1):1–9. DOI: 10.1016/j.autrev.2016.09.018.
10. Papo M., Sinico R.A., Teixeira V. et al. Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Rheumatology (Oxford). 2021;60(9):4355–4360. DOI: 10.1093/rheumatology/keaa805.
11. Liu S., Han L., Liu Y. et al. Clinical Significance of MPO-ANCA in Eosinophilic Granulomatosis With Polyangiitis: Experience From a Longitudinal Chinese Cohort. Front Immunol. 2022;13:885198. DOI: 10.3389/fimmu.2022.885198.
12. Chung S.A., Langford C.A., Maz M. et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Arthritis Rheumatol. 2021;73(8):1366–1383. DOI: 10.1002/art.41773.
13. Villa-Forte A. Eosinophilic granulomatosis with polyangiitis. Postgrad Med. 2023;135(sup1):52–60. DOI: 10.1080/00325481.2022.2134624.
14. Guillevin L., Lhote F., Gayraud M. et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore). 1996;75(1):17–28. DOI: 10.1097/00005792-199601000-00003.
15. Guillevin L., Pagnoux C., Seror R. et al. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 2011;90(1):19–27. DOI: 10.1097/MD.0b013e318205a4c6.
16. Emmi G., Bettiol A., Gelain E. et al. Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis. Nat Rev Rheumatol. 2023;19(6):378–393. DOI: 10.1038/s41584-023-00958-w.
17. Войцеховский В.В., Погребная М.В., Гоборов Н.Д. и др. Особенности диагностики и лечения синдрома Черджа—Стросс. Бюллетень физиологии и патологии дыхания. 2017;64:79–87. DOI: 10.12737/article_59361a30e7f1e7.92279749. Voytsekhovskiy V.V., Pogrebnaya M.V., Goborov N.D. et al. Peculiarities of diagnosis and treatment of Churg-Strauss syndrome. Bulletin Physiology and Pathology of Respiration. 2017;64:79–87 (in Russ.). DOI: 10.12737/article_59361a30e7f1e7.92279749.
18. Баранов А.А. Системные васкулиты: современные стандарты диагностики и лечения. РМЖ. 2005;24:1577–1581. Baranov A.A. Systemic vasculitis: modern standards of diagnosis and treatment. RMJ. 2005;24:1577–1581 (in Russ.).
19. Бекетова Т.В. Европейские (EULAR/ERA-EDTA) рекомендации по диагностике и лечению АНЦА-ассоциированных системных васкулитов. Научно-практическая ревматология. 2017;55(1):12–16. DOI: 10.14412/1995-4484-2017-12-16. Beketova T.V. The 2016 European (EULAR/ERA-EDTA) recommendations for the diagnosis and management of ANCA-associated systemic vasculitis. Rheumatology Science and Practice. 2017;55(1):12–16 (in Russ.). DOI: 10.14412/1995-4484-2017-12-16.
20. Wilhelm A.B., Pinsky S., Ahmad S. et al. Endomyocardial biopsy facilitates diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA): a case report. Cardiovasc Pathol. 2022;58:107407. DOI: 10.1016/j.carpath.2022.107407.
21. Fijolek J., Radzikowska E. Eosinophilic granulomatosis with polyangiitis — Advances in pathogenesis, diagnosis, and treatment. Front Med (Lausanne). 2023;10:1145257. DOI: 10.3389/fmed.2023.1145257.
22. Mukhtyar C., Lee R., Brown D. et al. Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Ann Rheum Dis. 2009;68(12):1827–1832. DOI: 10.1136/ard.2008.101279.
23. Groh M., Pagnoux C., Baldini C. et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Intern Med. 2015;26(7):545–553. DOI: 10.1016/j.ejim.2015.04.022.
24. Puéchal X., Pagnoux C., Baron G. et al. Adding Azathioprine to Remission-Induction Glucocorticoids for Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss), Microscopic Polyangiitis, or Polyarteritis Nodosa Without Poor Prognosis Factors: A Randomized, Controlled Trial. Arthritis Rheumatol. 2017;69(11):2175–2186. DOI: 10.1002/art.40205.
25. Steinfeld J., Bradford E.S., Brown J. et al. Evaluation of clinical benefit from treatment with mepolizumab for patients with eosinophilic granulomatosis with polyangiitis. J Allergy Clin Immunol. 2019;143(6):2170–2177. DOI: 10.1016/j.jaci.2018.11.041.
26. Pavord I.D., Korn S., Howarth P. et al. Mepolizumab for severe eosinophilic asthma (DREAM): a multicentre, double-blind, placebo-controlled trial. Lancet. 2012;380(9842):651–659. DOI: 10.1016/S0140-6736(12)60988-X.
27. Bel E.H., Wenzel S.E., Thompson P.J. et al. Oral glucocorticoid-sparing effect of mepolizumab in eosinophilic asthma. N Engl J Med. 2014;371(13):1189–1197. DOI: 10.1056/NEJMoa1403291.
28. Bettiol A., Urban M.L., Dagna L. et al. Mepolizumab for Eosinophilic Granulomatosis With Polyangiitis: A European Multicenter Observational Study. Arthritis Rheumatol. 2022;74(2):295–306. DOI: 10.1002/art.41943.
29. Wechsler M.E., Akuthota P., Jayne D. et al. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med. 2017;376(20):1921–1932. DOI: 10.1056/NEJMoa1702079.
This work is licensed under a Creative Commons «Attribution» 4.0 License.
Previous article
Next article
