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A Case of ANCA-Positive Vasculitis Without Lung Involvement: Eosinophilic Granulomatous Polyangiitis (EGPA)

Year 2025, Volume: 11 Issue: 1, 131 - 135

Abstract

ABSTRACT
Eosinophilic Granulomatous Polyangiitis (EGPA) is a vasculitic disease with multiple organ involvement, allergic rhinitis and asthma components. A 60-year-old male patient was admitted to our clinic with constitutional symptoms such as weakness and fatigue, persistent asthma and acute renal injury (ARI). He stated that he had widespread myalgia, night sweats, and occasional rashes on the body at the time of admission. A diagnosis of Eosinophilic Granulomatous Polyangiitis was made based on clinical and laboratory evaluations of the patient, who received multi-stage therapy for asthma and allergic rhinitis. We present this patient, who is quite rare, affects small and medium-sized vessels, and is among ANCA positive vasculitides such as Granulomatosis with Polyangiitis (GPA) and microscopic polyangiitis, to discuss with the literature.

References

  • 1. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CG, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DG, Specks U, Stone JH, Takahashi K, Watts RA. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65(1):1-11.
  • 2. Trivioli G, Terrier B, Vaglio A. Eosinophilic granulomatosis with polyangiitis: understanding the disease and its management. Rheumatology (Oxford). 2020; 59(Suppl 3):iii84-iii94.
  • 3. Sablé-Fourtassou R, Cohen P, Mahr A, Pagnoux C, Mouthon L, Jayne D, Blockmans D, Cordier JF, Delaval P, Puechal X, Lauque D, Viallard JF, Zoulim A, Guillevin L; French Vasculitis Study Group. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med 2005; 143(9):632-8.
  • 4. Comarmond C, Pagnoux C, Khellaf M, Cordier JF, Hamidou M, Viallard JF, Maurier F, Jouneau S, Bienvenu B, Puéchal X, Aumaître O, Le Guenno G, Le Quellec A, Cevallos R, Fain O, Godeau B, Seror R, Dunogué B, Mahr A, Guilpain P, Cohen P, Aouba A, Mouthon L, Guillevin L; 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(1):270-81.
  • 5. Moosig F, Bremer JP, Hellmich B, Holle JU, Holl-Ulrich K, Laudien M, Matthis C, Metzler C, Nölle B, Richardt G, Gross WL. A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients. Ann Rheum Dis 2013; 72(6):1011-7.
  • 6. Sinico RA, Di Toma L, Maggiore U, Bottero P, Radice A, Tosoni C, Grasselli C, Pavone L, Gregorini G, Monti S, Frassi M, Vecchio F, Corace C, Venegoni E, Buzio C. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum 2005; 52(9):2926-35.
  • 7. Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore) 1999; 78(1):26-37.
  • 8. Bacciu A, Bacciu S, Mercante G, Ingegnoli F, Grasselli C, Vaglio A, Pasanisi E, Vincenti V, Garini G, Ronda N, Ferri T, Corradi D, Buzio C. Ear, nose and throat manifestations of Churg-Strauss syndrome. Acta Otolaryngol 2006; 126(5):503-9.
  • 9. Valent P, Klion AD, Horny HP, Roufosse F, Gotlib J, Weller PF, Hellmann A, Metzgeroth G, Leiferman KM, Arock M, Butterfield JH, Sperr WR, Sotlar K, Vandenberghe P, Haferlach T, Simon HU, Reiter A, Gleich GJ. Contemporary consensus proposal on criteria and classification of eosinophilic disorders and related syndromes. J Allergy Clin Immunol 2012; 130(3):607-612.e9.
  • 10. Jeong YJ, Kim KI, Seo IJ, Lee CH, Lee KN, Kim KN, Kim JS, Kwon WJ. Eosinophilic lung diseases: a clinical, radiologic, and pathologic overview. Radiographics 2007; 27(3):617-37; discussion 637-9.
  • 11. Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore). 1984; 63(2):65-81.
  • 12. Sinico RA, Di Toma L, Maggiore U, Tosoni C, Bottero P, Sabadini E, Giammarresi G, Tumiati B, Gregorini G, Pesci A, Monti S, Balestrieri G, Garini G, Vecchio F, Buzio C. Renal involvement in Churg-Strauss syndrome. Am J Kidney Dis 2006; 47(5):770-9.
  • 13. Cattaneo L, Chierici E, Pavone L, Grasselli C, Manganelli P, Buzio C, Pavesi G. Peripheral neuropathy in Wegener's granulomatosis, Churg-Strauss syndrome and microscopic polyangiitis. J Neurol Neurosurg Psychiatry 2007; 78(10):1119-23.
  • 14. Guilpain P, Auclair JF, Tamby MC, Servettaz A, Mahr A, Weill B, Guillevin L, Mouthon L. Serum eosinophil cationic protein: a marker of disease activity in Churg-Strauss syndrome. Ann N Y Acad Sci 2007; 1107:392-9.
  • 15. Inamo J, Kaneko Y, Ota Y, Takeuchi T. Subtypes in eosinophilic granulomatosis with polyangiitis classified according to rheumatoid factor. Clin Rheumatol 2019; 38(12):3493-9.
  • 16. Sablé-Fourtassou R, Cohen P, Mahr A, Pagnoux C, Mouthon L, Jayne D, Blockmans D, Cordier JF, Delaval P, Puechal X, Lauque D, Viallard JF, Zoulim A, Guillevin L; French Vasculitis Study Group. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med 2005; 143(9):632-8.
  • 17. Bacciu A, Buzio C, Giordano D, Pasanisi E, Vincenti V, Mercante G, Grasselli C, Bacciu S. Nasal polyposis in Churg-Strauss syndrome. Laryngoscope 2008 ; 118(2):325-9.

Akciğer Tutulumu Olmayan Bir ANCA Pozitif Vaskülit Vakası: Eozinofilik Granülomatöz Polianjitis (EGPA)

Year 2025, Volume: 11 Issue: 1, 131 - 135

Abstract

ÖZ
Eozinofilik Granülomatöz Polianjitis (EGPA) çoklu organ tutulumu ile seyreden alerjik rinit ve astım komponenti olan vaskülitik bir hastalıktır. Altmış yaşında erkek hasta kliniğimize dirençli astım, halsizlik yorgunluk gibi konstitüsyonel semptom ve akut böbrek hasarı (ABH) tanısı ile interne edildi. Başvuru esnasında yaygın miyalji, gece terlemesi ,vücutta ara ara ortaya çıkan döküntülerin olduğunu ifade ediyordu. Astım ve alerjik rinit nedeniyle çoklu basamak tedavisi alan hastaya yapılan klinik ve laboratuvar değerlendirmelerle Eozinofilik Granülomatöz Polianjitis tanısı konuldu. Oldukça nadir görülen, küçük ve orta dereceli büyüklükteki damarları tutan ve Granülomatosizli Polianjitis (GPA) ve mikroskobik polianjitis gibi ANCA pozitif vaskülitler arasında yer alan bu hastayı literatür eşliğinde tartışmak için sunuyoruz.

References

  • 1. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CG, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DG, Specks U, Stone JH, Takahashi K, Watts RA. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65(1):1-11.
  • 2. Trivioli G, Terrier B, Vaglio A. Eosinophilic granulomatosis with polyangiitis: understanding the disease and its management. Rheumatology (Oxford). 2020; 59(Suppl 3):iii84-iii94.
  • 3. Sablé-Fourtassou R, Cohen P, Mahr A, Pagnoux C, Mouthon L, Jayne D, Blockmans D, Cordier JF, Delaval P, Puechal X, Lauque D, Viallard JF, Zoulim A, Guillevin L; French Vasculitis Study Group. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med 2005; 143(9):632-8.
  • 4. Comarmond C, Pagnoux C, Khellaf M, Cordier JF, Hamidou M, Viallard JF, Maurier F, Jouneau S, Bienvenu B, Puéchal X, Aumaître O, Le Guenno G, Le Quellec A, Cevallos R, Fain O, Godeau B, Seror R, Dunogué B, Mahr A, Guilpain P, Cohen P, Aouba A, Mouthon L, Guillevin L; 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(1):270-81.
  • 5. Moosig F, Bremer JP, Hellmich B, Holle JU, Holl-Ulrich K, Laudien M, Matthis C, Metzler C, Nölle B, Richardt G, Gross WL. A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients. Ann Rheum Dis 2013; 72(6):1011-7.
  • 6. Sinico RA, Di Toma L, Maggiore U, Bottero P, Radice A, Tosoni C, Grasselli C, Pavone L, Gregorini G, Monti S, Frassi M, Vecchio F, Corace C, Venegoni E, Buzio C. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum 2005; 52(9):2926-35.
  • 7. Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore) 1999; 78(1):26-37.
  • 8. Bacciu A, Bacciu S, Mercante G, Ingegnoli F, Grasselli C, Vaglio A, Pasanisi E, Vincenti V, Garini G, Ronda N, Ferri T, Corradi D, Buzio C. Ear, nose and throat manifestations of Churg-Strauss syndrome. Acta Otolaryngol 2006; 126(5):503-9.
  • 9. Valent P, Klion AD, Horny HP, Roufosse F, Gotlib J, Weller PF, Hellmann A, Metzgeroth G, Leiferman KM, Arock M, Butterfield JH, Sperr WR, Sotlar K, Vandenberghe P, Haferlach T, Simon HU, Reiter A, Gleich GJ. Contemporary consensus proposal on criteria and classification of eosinophilic disorders and related syndromes. J Allergy Clin Immunol 2012; 130(3):607-612.e9.
  • 10. Jeong YJ, Kim KI, Seo IJ, Lee CH, Lee KN, Kim KN, Kim JS, Kwon WJ. Eosinophilic lung diseases: a clinical, radiologic, and pathologic overview. Radiographics 2007; 27(3):617-37; discussion 637-9.
  • 11. Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore). 1984; 63(2):65-81.
  • 12. Sinico RA, Di Toma L, Maggiore U, Tosoni C, Bottero P, Sabadini E, Giammarresi G, Tumiati B, Gregorini G, Pesci A, Monti S, Balestrieri G, Garini G, Vecchio F, Buzio C. Renal involvement in Churg-Strauss syndrome. Am J Kidney Dis 2006; 47(5):770-9.
  • 13. Cattaneo L, Chierici E, Pavone L, Grasselli C, Manganelli P, Buzio C, Pavesi G. Peripheral neuropathy in Wegener's granulomatosis, Churg-Strauss syndrome and microscopic polyangiitis. J Neurol Neurosurg Psychiatry 2007; 78(10):1119-23.
  • 14. Guilpain P, Auclair JF, Tamby MC, Servettaz A, Mahr A, Weill B, Guillevin L, Mouthon L. Serum eosinophil cationic protein: a marker of disease activity in Churg-Strauss syndrome. Ann N Y Acad Sci 2007; 1107:392-9.
  • 15. Inamo J, Kaneko Y, Ota Y, Takeuchi T. Subtypes in eosinophilic granulomatosis with polyangiitis classified according to rheumatoid factor. Clin Rheumatol 2019; 38(12):3493-9.
  • 16. Sablé-Fourtassou R, Cohen P, Mahr A, Pagnoux C, Mouthon L, Jayne D, Blockmans D, Cordier JF, Delaval P, Puechal X, Lauque D, Viallard JF, Zoulim A, Guillevin L; French Vasculitis Study Group. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med 2005; 143(9):632-8.
  • 17. Bacciu A, Buzio C, Giordano D, Pasanisi E, Vincenti V, Mercante G, Grasselli C, Bacciu S. Nasal polyposis in Churg-Strauss syndrome. Laryngoscope 2008 ; 118(2):325-9.
There are 17 citations in total.

Details

Primary Language Turkish
Subjects Clinical Sciences
Journal Section Case Report
Authors

Latif Emre Özdemir 0000-0003-1086-7639

Laleş Yanık Göktürk 0000-0003-4424-4384

Orhan Ayyıldız 0000-0001-5673-8408

Early Pub Date January 20, 2025
Publication Date
Submission Date February 1, 2023
Published in Issue Year 2025 Volume: 11 Issue: 1

Cite

Vancouver Özdemir LE, Yanık Göktürk L, Ayyıldız O. Akciğer Tutulumu Olmayan Bir ANCA Pozitif Vaskülit Vakası: Eozinofilik Granülomatöz Polianjitis (EGPA). Akd Med J. 2025;11(1):131-5.