ENT Manifestations Revealing Wegener's Granulomatosis: Case Report
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Granulomatosis with polyangiitis, ENT involvement, antineutrophil cytoplasmic antibody (ANCA)
Abstract
Granulomatosis with polyangiitis, formerly known as Wegener granulomatosis, is a rare multisystem autoimmune disease of unknown etiology. It is one of the antineutrophil cytoplasmic antibody (ANCA) associated vasculitic disorders.
A 34-year-old male consulted in ENT department for bilateral purulent rhinorrhea, right hemicrania and dysphagia without fiver. He presented nasal saddle, complete destruction of nasal septum with otological and laryngological signs. These characteristic lesions pushed us to look for other systemic signs. We identify cranial nerve palsies and pulmonary damage. Nasal cavity biopsy with immunohistochemical study objectived nonspecific granulomatous inflammatory process. Tests for ANCA was positive. Typical ENT and pulmonary manifestations, histological results as well as a positive ANCA test allowed the diagnosis of Granulomatosis with polyangiitis. Treatment included mostly corticosteroid therapy (prednisone) with biotherapy (rituximab) and evolution was stationary without the occurrence of other complications.
In this manuscript, we try to describe this case in order to highlight the frequent ENT involvement in granulomatosis with polyangiitis and remind that it can reveal this disease.
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References
Garlapati P, Qurie A. Granulomatosis With Polyangiitis. StatPearls Publishing ; 2023 Jan-.
Iannella G, Greco A, Granata G, Manno A, Pasquariello B, Angeletti D, et al. Granulomatosis with polyangiitis and facial palsy: Literature review and insight in the autoimmune pathogenesis. Autoimmun Rev. 2016 Jul; 15(7):621-31.
Comarmond C, Cacoub P. Granulomatosis with polyangiitis (Wegener): clinical aspects and treatment. Autoimmun Rev. 2014 Nov;13(11):1121-5. [PubMed]
Ntatsaki E, Watts RA, Scott DG. Epidemiology of ANCA-associated vasculitis. Rheum Dis Clin North Am. 2010 Aug; 36(3):447-61. [PubMed]
Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, Calabrese LH, Fries JF, Lie JT, Lightfoot RW. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum. 1990 Aug;33(8):1101-7. [PubMed]
DeRemee RA, McDonald TJ, Harrison EG, Coles DT. Wegener's granulomatosis. Anatomic correlates, a proposed classification. Mayo Clin Proc. 1976 Dec; 51(12):777-81. [PubMed]
Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 1992; 116: 488–98.
Luis Fabris J, Jaramillo G, Torres R, Rosselli C, Olivares C. Renal compromise in a patient with Wegener's granulomatosis: clinical case and literature review. Revista Colombiana de Nefrología, vol. 4, no. 1, pp. 82-94, 2017
Monteiro M,Domingos R, Rocha S, Miranda I. Granulomatosis With Polyangiitis: The Complexity of Clinical Manifestations, Therapeutic Challenges, and Complications of a Severe Multisystemic Case. Cureus. 2023 Oct; 15(10): e47031.
Sada K-e, Yamamura M, Harigai M, Fujii T, Takasaki Y, Amano K, et al. Different responses to treatment across classified diseases and severities in Japanese patients with microscopic polyangiitis and granulomatosis with polyangiitis: a nationwide prospective inception cohort study. Arthritis Res Ther. 2015 ; 17(1):1-12.
Pinching AJ, Lockwood CM, Pussell BA, Rees AJ, Sweny P, Evans DJ, Bowley N, Peters DK. Wegener's granulomatosis: observations on 18 patients with severe renal disease. Q J Med. 1983 Autumn;52(208):435-60. [PubMed]