Allergy, Asthma & Respiratory Disease

The Korean Academy of Asthma, Allergy and Clinical Immunology (대한천식알레르기학회)
권호 표지
DOI QR코드

DOI QR Code

Eosinophilic granulomatosis with polyangiitis presenting as an endobronchial nodule and atelectasis: A case report

기관지 내 결절과 무기폐로 발현한 호산구 육아종증 다발혈관염 1예

  • Wan Ho Yoo (Department of Internal Medicine, Pusan National University School of Medicine) ;
  • Won Jin Lee (Department of Internal Medicine, Pusan National University School of Medicine) ;
  • Eun-Jung Jo (Department of Internal Medicine, Pusan National University School of Medicine) ;
  • Hye-Kyung Park (Department of Internal Medicine, Pusan National University School of Medicine)
  • 류완호 (부산대학교 의과대학 내과학교실) ;
  • 이원진 (부산대학교 의과대학 내과학교실) ;
  • 조은정 (부산대학교 의과대학 내과학교실) ;
  • 박혜경 (부산대학교 의과대학 내과학교실)
  • Received : 2024.03.31
  • Accepted : 2024.04.30
  • Published : 2024.07.30
Download PDF
⟨ Previous Next ⟩

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, multisystemic, small-to-medium vessel vasculitis characterized by asthma, blood and tissue eosinophilia. Pulmonary manifestations in EGPA are variable; however, endobronchial lesions without parenchymal involvement are rare. Herein, we describe a case of EGPA presenting as atelectasis and endobronchial nodule, which was confirmed by bronchoscopic biopsy. A 43-year-old woman with a history of asthma presented with fever, cough, and sputum. Chest computed tomography scan revealed an endobronchial nodule and total atelectasis in the right middle lobe. Bronchoscopy revealed a whitish nodular lesion blocking the right middle lobe bronchus at the origin of the right middle lobe bronchus. A bronchial mucosal biopsy specimen revealed chronic inflammation with eosinophilic angiitis and luminal eosinophilic abscess admixed with mucus. Clinical presentation and pathological results of bronchoscopic biopsy were consistent with EGPA. All symptoms and chest radiographic findings improved after initiating glucocorticoids.

Keywords

Acknowledgement

This work was supported by a 2-Year Research Grant of Pusan National University.

References

  1. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951;27:277-301.
  2. Gioffredi A, Maritati F, Oliva E, Buzio C. Eosinophilic granulomatosis with polyangiitis: an overview. Front Immunol 2014;5:549.
  3. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013;65:1-11.
  4. Comarmond C, Pagnoux C, Khellaf M, Cordier JF, Hamidou M, Viallard JF, et al. 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;65:270-81.
  5. Berti A, Boukhlal S, Groh M, Cornec D. Eosinophilic granulomatosis with polyangiitis: the multifaceted spectrum of clinical manifestations at different stages of the disease. Expert Rev Clin Immunol 2020;16:51-61.
  6. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990;33:1094-100.
  7. Sinico RA, Di Toma L, Maggiore U, Bottero P, Radice A, Tosoni C, et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum 2005;52:2926-35.
  8. Kim YK, Lee KS, Chung MP, Han J, Chong S, Chung MJ, et al. Pulmonary involvement in Churg-Strauss syndrome: an analysis of CT, clinical, and pathologic findings. Eur Radiol 2007;17:3157-65.
  9. Cottin V, Bel E, Bottero P, Dalhoff K, Humbert M, Lazor R, et al. Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Straus). Eur Respir J 2016;48:1429-41.
  10. Matsushima H, Takayanagi N, Kurashima K, Tokunaga D, Ubukata M, Kawabata Y, et al. Multiple tracheobronchial mucosal lesions in two cases of Churg-Strauss syndrome. Respirology 2006;11:109-12.
  11. Hara Y, Kanoh S, Shinkai M, Kawana A. Churg-Strauss syndrome with endobronchial eosinophilic vasculitis. Intern Med 2012;51:3227.
  12. Chen GL, Wu CH, Perng WC. Endobronchial lesion in eosinophilic granulomatosis with polyangiitis. Iran J Allergy Asthma Immunol 2017;16:561-4.
  13. Boo SJ, Lee K, Ra SW, Jin YJ, Park GM, Hong SB. A case of Churg-Strauss syndrome with multiple tracheobronchial mucosal lesions. Tuberc Respir Dis 2008;65:405-9.
  14. Maranini B, Guzzinati I, Casoni GL, Ballotta M, Lo Monaco A, Govoni M. Case report: middle lobe syndrome: a rare presentation in eosinophilic granulomatosis with polyangiitis. Front Immunol 2023;14:1222431.
  15. Agarwal R, Chakrabarti A, Shah A, Gupta D, Meis JF, Guleria R, et al. Allergic bronchopulmonary aspergillosis: review of literature and proposal of new diagnostic and classification criteria. Clin Exp Allergy 2013;43:850-73.
  16. Hebisawa A, Tamura A, Kurashima A, Oobayashi C, Kawamata M, Maeda M, et al. Pathologic reconsideration on allergic bronchopulmonary aspergillosis and mycosis. Nihon Kokyuki Gakkai Zasshi 1998;36:330-7.
  17. Sacoto G, Boukhlal S, Specks U, Flores-Suarez LF, Cornec D. Lung involvement in ANCA-associated vasculitis. Presse Med 2020;49:104039.
  18. Polychronopoulos VS, Prakash UBS, Golbin JM, Edell ES, Specks U. Airway Involvement in Wegener's granulomatosis. Rheum Dis Clin North Am 2007;33:755-75, vi.
  19. Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore) 2011;90:19-27.
  20. Ribi C, Cohen P, Pagnoux C, Mahr A, Arene JP, Lauque D, et al. Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients. Arthritis Rheum 2008;58:586-94.