EN PL
PRACA PRZEGLĄDOWA
 
SŁOWA KLUCZOWE
DZIEDZINY
STRESZCZENIE
Introduction:
Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by eosinophilic granulomatous vasculitis. Typical symptoms include late-onset bronchial asthma and blood and tissue eosinophilia. In addition to these characteristic symptoms, EGPA can affect important organs such as the skin, kidneys, heart, sinuses, gastrointestinal tract, and nervous system. Given the variability of the clinical presentation, EGPA is challenging to diagnose. Furthermore, EGPA often occurs in phases, with clinical manifestations and pathological findings varying depending on the affected anatomic site and stage of disease.

Material and Methods:
The authors reviewed the SCOPUS, MEDLINE, and PubMed medical databases to prepare an overview of the clinical manifestations and diagnosis for EGPA.

Results:
This comprehensive review examines the current knowledge on the clinical course of EGPA, diagnostic options and prognostic factors.

Conclusions:
We highlight the diverse organ involvement observed in EGPA, particularly in association with eosinophilic and vasculitic manifestations. Our findings underscore the importance of anti- neutrophil cytoplasm antibody status as a potential key factor influencing disease presentation.

REFERENCJE (53)
1.
White J, Dubey S. Eosinophilic granulomatosis with polyangiitis: a review. Autoimmun Rev 2023; 22: 103219, DOI: 10.1016/ j.autrev.2022.103219.
 
2.
Furuta S, Iwamoto T, Nakajima H. Update on eosinophilic granulomatosis with polyangiitis. Allergol Int 2019; 68: 430–436, DOI: 10.1016/j.alit.2019.06.004.
 
3.
Chen Y, Ding Y, Liu Z, et al. Long-term outcomes in antineutrophil cytoplasmic autoantibody-positive eosinophilic granulomatosis with polyangiitis patients with renal involvement: a retrospective study of 14 Chinese patients. BMC Nephrol 2016; 17: 101, DOI: 10.1186/s12882-016-0319-2.
 
4.
Reggiani F, L’Imperio V, Calatroni M, et al. Renal involvement in eosinophilic granulomatosis with polyangiitis. Front Med (Lausanne) 2023; 10: 1244651, DOI: 10.3389/fmed.2023.
 
5.
Drynda A, Padjas A, Wójcik K, et al. Clinical Characteristics of EGPA Patients in Comparison to GPA Subgroup with Increased Blood Eosinophilia from POLVAS Registry. J Immunol Res 2024; 2024: 4283928, DOI: 10.1155/2024/4283928.
 
6.
Tan JA, Dehghan N, Chen W, et al. Mortality in ANCA-associat­ed vasculitis: a meta-analysis of observational studies. Ann Rheum Dis 2017; 76: 1566–1574, DOI: 10.1136/annrheumdis- 2016-210942.
 
7.
Pyo JY, Lee LE, Park YB, Lee SW. Comparison of the 2022 ACR/EULAR Classification Criteria for Antineutrophil Cytoplasmic Antibody-Associated Vasculitis with Previous Criteria. Yonsei Med J 2023; 64: 11–17, DOI: 10.3349/ymj.2022.0435.
 
8.
Grayson PC, 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: 309–314, DOI: 10.1136/annrheumdis-2021-221794.
 
9.
Valent P, Klion AD, Roufosse F, et al. Proposed refined diagnostic criteria and classification of eosinophil disorders and related syndromes. Allergy 2023; 78: 47–59, DOI: 10.1111/all.15544.
 
10.
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.
 
11.
Klion AD, Bochner BS, Gleich GJ, et al.; Hypereosinophilic Syndromes Working Group. Approaches to the treatment of hyper­eosinophilic syndromes: a workshop summary report. J Allergy Clin Immunol 2006; 117: 1292–1302, DOI: 10.1016/j.jaci.2006.02.042.
 
12.
Roufosse FE, Goldman M, Cogan E. Hypereosinophilic syndromes. Orphanet J Rare Dis 2007; 2: 37, DOI: 10.1186/1750-1172-2-37.
 
13.
Khoury P, Zagallo P, Talar-Williams C, et al. Serum biomarkers are similar in Churg-Strauss syndrome and hypereosinophilic syndrome. Allergy 2012; 67: 1149–1156, DOI: 10.1111/j.1398-9995.2012.02873.x.
 
14.
Innocenti A, Melita D, Ghezzi S. Re: Sarah R. Ottenhof, Andrew Leone, Rosa S. Djajadiningrat, et al. Surgical and Oncological Outcomes in Patients After Vascularized Flap Reconstruction for Locoregionally Advanced Penile Cancer. Eur Urol Focus. In press. DOI: 10.1016/j.euf.2018.02.002. Eur Urol Focus 2019; 5: 1157, DOI: 10.1016/j.euf.2018.04.007.
 
15.
Wechsler ME, Hellmich B, Cid MC, et al. Unmet needs and evi­dence gaps in hypereosinophilic syndrome and eosinophilic granulomatosis with polyangiitis. J Allergy Clin Immunol 2023; 151: 1415–1428, DOI: 10.1016/j.jaci.2023.03.011.
 
16.
Cottin V, Bel E, Bottero P, et al. Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Eur Respir J 2016; 48: 1429–1441, DOI: 10.1183/13993003.00097-2016.
 
17.
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: 545–553, DOI: 10.1016/j.ejim. 2015.04.022.
 
18.
Choi YH, Im JG, Han BK, et al. Thoracic manifestation of Churg-Strauss syndrome: radiologic and clinical findings. Chest 2000; 117: 117–124, DOI: 10.1378/chest.117.1.117.
 
19.
Cordier JF, Cottin V, Khouatra C, et al. Hypereosinophilic obli­terative bronchiolitis: a distinct, unrecognised syndrome. Eur Respir J 2013; 41: 1126–1134, DOI: 10.1183/09031936.00099812.
 
20.
Fouka E, Drakopanagiotakis F, Steiropoulos P. Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome. Int J Mol Sci 2024; 25: 5278, DOI: 10.3390/ijms25105278.
 
21.
Greco A, Rizzo MI, De Virgilio A, et al. Churg-Strauss syndrome. Autoimmun Rev 2015; 14: 341–348, DOI: 10.1016/j.autrev. 2014.12.004.
 
22.
Wu EY, Hernandez ML, Jennette JC, Falk RJ. Eosinophilic Granulomatosis with Polyangiitis: Clinical Pathology Conference and Review. J Allergy Clin Immunol Pract 2018; 6: 1496–1504, DOI: 10.1016/j.jaip.2018.07.001.
 
23.
Neumann T, Manger B, Schmid M, et al. Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis. Medicine (Baltimore) 2009; 88: 236–243, DOI: 10.1097/MD. 0b013e3181af35a5.
 
24.
Bond M, Fagni F, Moretti M, et al. At the Heart of Eosinophilic Granulomatosis with Polyangiitis: into Cardiac and Vascular Involvement. Curr Rheumatol Rep 2022; 24: 337–351, DOI: 10.1007/s11926-022-01087-1.
 
25.
Mahr A, Moosig F, Neumann T, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): evolutions in classification, etiopathogenesis, assessment and management. Curr Opin Rheumatol 2014; 26: 16–23, DOI: 10.1097/BOR. 0000000000000015.
 
26.
Liu X, Zhou Y, Li J, et al. Cardiac involvement in eosinophilic granulomatosis with polyangiitis: acute eosinophilic myocarditis and chronic inflammatory cardiomyopathy. Rheumatology (Oxford) 2024; 2024: keae085, DOI: 10.1093/rheumatology/keae085.
 
27.
Vallianou NG, Geladari E, Panagopoulos F, Kalantzi M. Cardiac MRI in Autoimmune Diseases: Where Are We Now? Curr Cardiol Rev 2021; 17: e160721190002, DOI: 10.2174/1573403X16666210108104236.
 
28.
Comarmond C, Pagnoux C, Khellaf M, et al. French Vasculitis Study Group. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum 2013; 65: 270–281, DOI: 10.1002/art.37721.
 
29.
Bridges C, Shenk MER, Martin K, Launhardt A. Cutaneous manifestations of childhood Eosinophilic Granulomatosis with Polyangiitis (cEGPA): a case-based review. Pediatr Dermatol 2020; 37: 604–612, DOI: 10.1111/pde.14144.
 
30.
Kawakami T, Soma Y, Kawasaki K, et al. Initial cutaneous manifestations consistent with mononeuropathy multiplex in Churg-Strauss syndrome. Arch Dermatol 2005; 141: 873–878, DOI: 10.1001/archderm.
 
31.
Seccia V, Fortunato S, Cristofani-Mencacci L, et al. Focus on audiologic impairment in eosinophilic granulomatosis with polyangiitis. Laryngoscope 2016; 126: 2792–2797, DOI: 10.1002/lary.25964.
 
32.
Low CM, Keogh KA, Saba ES, et al. Chronic rhinosinusitis in eosinophilic granulomatosis with polyangiitis: clinical presentation and antineutrophil cytoplasmic antibodies. Int Forum Allergy Rhinol 2020; 10: 217–222, DOI: 10.1002/alr.22503.
 
33.
Iwata M, Fukutomi Y, Hamada Y, et al. Computed tomography findings of paranasal sinuses in patients with eosinophilic granulomatosis with polyangiitis: Comparison with other eosinophilic sinus diseases and clinical relevance of their severity. Allergol Int 2023; 72: 428–436, DOI: 10.1016/j.alit.2023.02.003.
 
34.
Bacciu A, Buzio C, Giordano D, et al. Nasal polyposis in Churg-Strauss syndrome. Laryngoscope 2008; 118: 325–329, DOI: 10.1097/MLG.0b013e318159889d.
 
35.
Pagnoux C, Mahr A, Cohen P, Guillevin L. Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome, or rheumatoid arthritis-associated vasculitis. Medicine (Baltimore) 2005; 84: 115–128, DOI: 10.1097/01.md.0000158825.87055.0b.
 
36.
Li R, Chen Y, Zhang S, et al. Clinical characteristics and long-term outcome of patients with gastrointestinal involvement in eosinophilic granulomatosis with polyangiitis. Front Immunol 2023; 13: 1099722, DOI: 10.3389/fimmu.2022.1099722.
 
37.
Arnold S, Mahrhold J, Kerstein-Staehle A, et al. Spectrum of ANCA-specificities in eosinophilic granulomatosis with polyangiitis. A retrospective multicentre study. Clin Exp Rheumatol 2023; 41: 936–942, DOI: 10.55563/clinexprheumatol/299yme.
 
38.
Hokama A, Kishimoto K, Ihama Y, et al. Endoscopic and radiographic features of gastrointestinal involvement in vasculitis. World J Gastrointest Endosc 2012; 4: 50–56, DOI: 10.4253/wjge.v4.i3.50.
 
39.
Kronbichler A, Shin JI, Lee KH, et al. Clinical associations of renal involvement in ANCA-associated vasculitis. Autoimmun Rev 2020; 19: 102495, DOI: 10.1016/j.autrev.2020.102495.
 
40.
Sinico RA, Di Toma L, Maggiore U, et al. Renal involvement in Churg-Strauss syndrome. Am J Kidney Dis 2006; 47: 770–779, DOI: 10.1053/j.ajkd.2006.01.026.
 
41.
Durel CA, Sinico RA, Teixeira V, et al. French Vasculitis Study Group (FVSG). Renal involvement in eosinophilic granulomatosis with polyangiitis (EGPA): a multicentric retrospective study of 63 biopsy-proven cases. Rheumatology (Oxford) 2021; 60: 359–365, DOI: 10.1093/rheumatology/keaa416.
 
42.
Brix SR, Noriega M, Tennstedt P, et al. Development and vali­dation of a renal risk score in ANCA-associated glomerulonephritis. Kidney Int 2018; 94: 1177–1188, DOI: 10.1016/j.kint. 2018.07.020.
 
43.
Challa A, Sirisha S, Khandelia H, et al. A case of eosinophilic granulomatous polyangiitis with concurrent central and peripheral nervous system involvement. Oxf Med Case Reports 2023; 2023: omad067, DOI: 10.1093/omcr/omad067.
 
44.
Zhang Z, Liu S, Guo L, et al. Clinical Characteristics of Peri­pheral Neuropathy in Eosinophilic Granulomatosis with Polyangiitis: A Retrospective Single-Center Study in China. J Immunol Res 2020; 2020: 3530768, DOI: 10.1155/2020/3530768.
 
45.
Liu S, Guo L, Fan X, et al. Clinical features of central nervous system involvement in patients with eosinophilic granulomatosis with polyangiitis: a retrospective cohort study in China. Orphanet J Rare Dis 2021; 16: 152, DOI: 10.1186/s13023-021-01780-x.
 
46.
Nie N, Liu L, Bai C, et al. Eosinophilic granulomatous polyangiitis with central nervous system involvement in children: a case report and literature review. Front Immunol 2024; 15: 1406424, DOI: 10.3389/fimmu.2024.1406424.
 
47.
Allenbach Y, Seror R, Pagnoux C, et al. High frequency of venous thromboembolic events in Churg-Strauss syndrome, Wegener’s granulomatosis and microscopic polyangiitis but not polyarteritis nodosa: a systematic retrospective study on 1130 patients. Ann Rheum Dis 2009; 68: 564–567. DOI: 10.1136/ard.2008.099051.
 
48.
Bettiol A, Sinico RA, Schiavon F, et al. Risk of acute arterial and venous thromboembolic events in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Eur Respir J 2021; 57: 2004158, DOI: 10.1183/13993003.04158-2020.
 
49.
Kanno K, Minami-Hori M, Honma M, Ishida-Yamamoto A. Histopathological Findings and Increased D-Dimer Are Predictive Factors of Systemic Thromboses in Eosinophilic Granu­lomatosis With Polyangiitis. Am J Dermatopathol 2018; 40: 879–883, DOI: 10.1097/DAD.0000000000001202.
 
50.
Saito Y, Okada S, Funabashi N, Kobayashi Y. ANCA-negative eosinophilic granulomatosis with polyangitis (EGPA) manifesting as a large intracardiac thrombus and glomerulonephritis with angionecrosis. BMJ Case Rep 2016; 2016: bcr2016216520, DOI: 10.1136/bcr-2016-216520.
 
51.
Wolf F, Glick K, Elias M, Mader R. Portal Vein Thrombosis and Thrombocytopenia in Eosinophilic Granulomatosis with Polyangiitis: A Paradox? Eur J Case Rep Intern Med 2018; 5: 000971, DOI: 10.12890/2018_000971.
 
52.
Zhu D, Luo Y, Liu X, Zu L. Antiproteinase 3 Positive Eosinophilic Granulomatosis with Polyangiitis Presenting with Heart Failure and Intraventricular Thrombosis. Case Rep Rheumatol 2017; 2017: 2908185, DOI: 10.1155/2017/2908185.
 
53.
Jiang XD, Guo S, Zhang WM. Acute myocardial infarction induced by eosinophilic granulomatosis with polyangiitis: a case report. World J Clin Cases 2021; 9: 10702–10707, DOI: 10.12998/wjcc.v9.i34.10702.
 
Copyright: © Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie. This is an Open Access journal, all articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License (https://creativecommons.org/licenses/by-nc-sa/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
eISSN:2084-9834
ISSN:0034-6233
Journals System - logo
Scroll to top