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Current Rheumatology Reviews

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ISSN (Print): 1573-3971
ISSN (Online): 1875-6360

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Case Report

Atypical Debut of Granulomatosis with Polyangiitis as Acute Tonsilitis and Strawberry Gum: A Case Report

Author(s): Cristiana Sieiro Santos*, Clara Moriano Morales, Carolina Álvarez Castro and Elvira Diez Alvarez

Volume 19, Issue 1, 2023

Published on: 17 June, 2022

Page: [102 - 107] Pages: 6

DOI: 10.2174/1573397118666220413120904

Price: $65

Abstract

Abstract: Background: Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis characterized by necrosis, granulomatous inflammation, and vasculitis. It is characterized by the triad of the upper and lower respiratory system, lung, and kidney disease. Although it is usually a multisystemic disease, limited forms have also been described, and otolaryngological involvement is the first manifestation in up to 80-95% of the cases.

Case Presentation: In this report, we describe the case of an ANCA negative patient with a limited form of GPA that presented a necrotic lesion confined to the right tonsil compatible with granulomatosis with polyangiitis, which later presented positive ANCA antibodies. Oral lesions may be the initial manifestation of GPA, and systemic involvement can be presented within weeks or months. Although the oral manifestations have been well described, the initial presentation with oral lesions is very rare, and presentation with oropharyngeal manifestation is even rarer. This disease is generally characterized by anti-neutrophil cytoplasmic antibodies (ANCA); however, there are rare cases with negative ANCA.

Conclusion: The diagnosis was established based on the clinical presentation and the histopathological findings of the characteristic inflammatory pattern.

Keywords: Tonsillitis, Granulomatosis, Strawberry gum, Vasculitis, GPA, Fibrinoid necrosis, polyangiitis.

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Graphical Abstract

[1]
Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum 2013; 65(1): 1-11.
[http://dx.doi.org/10.1002/art.37715] [PMID: 23045170]
[2]
Silva-Fernández L, Loza E, Martínez-Taboada VM, Blanco R. From the systemic autoimmune diseases study group of the Spanish society for rheumatology (EAS-SER). Biological therapy for systemic vasculitis: A systematic review. Semin Arthritis Rheum 2014; 43: 542-57.
[PMID: 23978781]
[3]
Fienberg R. Necrotizing granulomatosis and angiitis of the lungs with massive splenic necrosis and focal thrombotic granulomatous glomerulonephritis. Am J Clin Pathol 1953; 23(5): 413-28.
[http://dx.doi.org/10.1093/ajcp/23.5.413] [PMID: 13040300]
[4]
Carrington CB, Liebow A. Limited forms of angiitis and granulomatosis of Wegener’s type. Am J Med 1966; 41(4): 497-527.
[http://dx.doi.org/10.1016/0002-9343(66)90214-2] [PMID: 5923596]
[5]
Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: An analysis of 158 patients. Ann Intern Med 1992; 116(6): 488-98.
[http://dx.doi.org/10.7326/0003-4819-116-6-488] [PMID: 1739240]
[6]
Reinhold-Keller E, Beuge N, Latza U, et al. An interdisciplinary approach to the care of patients with Wegener’s granulomatosis: Long-term outcome in 155 patients. Arthritis Rheum 2000; 43(5): 1021-32.
[http://dx.doi.org/10.1002/1529-0131(200005)43:5<1021::AID-ANR10>3.0.CO;2-J] [PMID: 10817555]
[7]
Jayne D. Update on the European vasculitis study group trials. Curr Opin Rheumatol 2001; 13(1): 48-55.
[http://dx.doi.org/10.1097/00002281-200101000-00008] [PMID: 11148715]
[8]
WGET Research Group. Design of the Wegener’s granulomatosis etanercept trial (WGET). Control Clin Trials 2002; 23(4): 450-68.
[http://dx.doi.org/10.1016/S0197-2456(02)00209-X] [PMID: 12161090]
[9]
Hellmich B, Flossmann O, Gross WL, et al. EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: Focus on anti-neutrophil cytoplasm antibody-associated vasculitis. Ann Rheum Dis 2007; 66(5): 605-17.
[http://dx.doi.org/10.1136/ard.2006.062711] [PMID: 17170053]
[10]
Wegener F. Über generalisierte, septische Gefässerkrankungen. Verh Dtsch Ges Pathol 1936; 29: 202-10.
[11]
McDonald TJ, DeRemee RA, Kern EB, Harrison EG Jr. Nasal manifestations of Wegener’s granulomatosis. Laryngoscope 1974; 84(12): 2101-12.
[http://dx.doi.org/10.1288/00005537-197412000-00002] [PMID: 4437252]
[12]
Cassan SM, Divertie MB, Hollenhorst RW, Harrison EG Jr. Pseudotumor of the orbit and limited Wegener’s granulomatosis. Ann Intern Med 1970; 72(5): 687-93.
[http://dx.doi.org/10.7326/0003-4819-72-5-687] [PMID: 5448097]
[13]
Ito Y, Shinogi J, Yuta A, Okada E, Taki M, Matsukage H. Clinical records: A case report of Wegener’s granulomatosis limited to the ear. Auris Nasus Larynx 1991; 18(3): 281-9.
[http://dx.doi.org/10.1016/S0385-8146(12)80264-1] [PMID: 1799332]
[14]
Kurita S, Hirano M. Wegener’s granulomatosis localised in the larynx: Report of a case. Auris Nasus Larynx 1983; 10: 97-104.
[http://dx.doi.org/10.1016/S0385-8146(83)80011-X]
[15]
Cassan SM, Coles DT, Harrison EG Jr. The concept of limited forms of Wegener’s granulomatosis. Am J Med 1970; 49(3): 366-79.
[http://dx.doi.org/10.1016/S0002-9343(70)80029-8] [PMID: 4917075]
[16]
Pamela MK, Lutalo D’Cruz, David P. Diagnosis and classification of granulomatosis with polyangiitis (aka Wegener’s granulomatosis). J Autoimmun 2014; 94-8.
[17]
Holle JU, Gross WL, Holl-Ulrich K, et al. Prospective long-term follow-up of patients with localised Wegener’s granulomatosis: Does it occur as persistent disease stage? Ann Rheum Dis 2010; 69(11): 1934-9.
[http://dx.doi.org/10.1136/ard.2010.130203] [PMID: 20511614]
[18]
Handlers JP, Waterman J, Abrams AM, Melrose RJ. Oral features of Wegener’s granulomatosis. Arch Otolaryngol 1985; 111(4): 267-70.
[http://dx.doi.org/10.1001/archotol.1985.00800060091015] [PMID: 3977759]
[19]
Singh S, Balakrishnan C, Bajan K, Bhaduri A. Wegener’s granulomatosis presenting as a tonsillar mass. J Assoc Physicians India 2009; 57: 603.
[PMID: 20209726]
[20]
Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med 1997; 337(21): 1512-23.
[http://dx.doi.org/10.1056/NEJM199711203372106] [PMID: 9366584]
[21]
Comarmond C, Cacoub P. Granulomatosis with polyangiitis (Wegener): Clinical aspects and treatment. Conference Autoimmun Rev 2014; 13: 1121-5.
[22]
de Guevara DL, Cerda F, Carreño MA, Piottante A, Bitar P. Update in the study of granulomatosis with polyangiitis (Wegener’s granulomatosis). Rev Chil Radiol 2019; 25(1): 26-34.
[http://dx.doi.org/10.4067/S0717-93082019000100026]
[23]
Morales-Angulo C, García-Zornoza R, Obeso-Agüera S, Calvo-Alén J, González-Gay MA. Ear, nose and throat manifestations of Wegener's granulomatosis (granulomatosis with polyangiitis). Acta Otorrinolaringol Esp 2012; 63(3): 206-11.
[http://dx.doi.org/10.1016/j.otorri.2011.12.002] [PMID: 22439922]
[24]
Duna GF, Galperin C, Hoffman GS. Wegener’s granulomatosis. Rheum Dis Clin North Am 1995; 21(4): 949-86.
[http://dx.doi.org/10.1016/S0889-857X(21)00480-4] [PMID: 8592744]
[25]
Wawrzycka K, Szczeklik K, Darczuk D, Lipska W, Szczeklik W, Musiał J. Strawberry gingivitis as the first manifestation of granulomatosis with polyangiitis. Pol Arch Med Wewn 2014; 124(10): 551-2.
[http://dx.doi.org/10.20452/pamw.2468] [PMID: 25369512]
[26]
Shi L. Anti-neutrophil cytoplasmic antibody-associated vasculitis: Prevalence, treatment, and outcomes. Rheumatol Int 2017; 37(11): 1779-88.
[http://dx.doi.org/10.1007/s00296-017-3818-y] [PMID: 28956128]
[27]
van der Woude FJ, Rasmussen N, Lobatto S, et al. Autoantibodies against neutrophils and monocytes: Tool for diagnosis and marker of disease activity in Wegener’s granulomatosis. Lancet 1985; 1(8426): 425-9.
[http://dx.doi.org/10.1016/S0140-6736(85)91147-X] [PMID: 2857806]
[28]
Rao JK, Allen NB, Feussner JR, Weinberger M. A prospective study of antineutrophil cytoplasmic antibody (c-ANCA) and clinical criteria in diagnosing Wegener’s granulomatosis. Lancet 1995; 346(8980): 926-31.
[http://dx.doi.org/10.1016/S0140-6736(95)91555-9] [PMID: 7564726]
[29]
Yates M, Watts RA, Bajema IM, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis 2016; 75(9): 1583-94.
[http://dx.doi.org/10.1136/annrheumdis-2016-209133] [PMID: 27338776]
[30]
Bossuyt X, Cohen Tervaert JW, Arimura Y, et al. Position paper: Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis. Nat Rev Rheumatol 2017; 13(11): 683-92.
[http://dx.doi.org/10.1038/nrrheum.2017.140] [PMID: 28905856]
[31]
Ponniah I, Shaheen A, Shankar KA, Kumaran MG. Wegener’s granulomatosis: The current understanding. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005; 100(3): 265-70.
[http://dx.doi.org/10.1016/j.tripleo.2005.04.018] [PMID: 16122651]
[32]
Greco A, Marinelli C, Fusconi M, et al. Clinic manifestations in granulomatosis with polyangiitis. Int J Immunopathol Pharmacol 2016; 29(2): 151-9.
[http://dx.doi.org/10.1177/0394632015617063]
[33]
Knight JM, Hayduk MJ, Summerlin DJ, Mirowski GW. “Strawberry” gingival hyperplasia: A pathognomonic mucocutaneous finding in Wegener granulomatosis. Arch Dermatol 2000; 136(2): 171-3.
[http://dx.doi.org/10.1001/archderm.136.2.171] [PMID: 10677091]
[34]
Hochberg MC, Gravallese EM, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH. Rheumatology. (7th ed.), Philadelphia: Elsevier 2019.
[35]
Lally L, Spiera R. Current landscape of antineutrophil cytoplasmic antibody-associated vasculitis: Classification, diagnosis, and treatment. Rheum Dis Clin North Am 2015; 41(1): 1-19. vii.
[http://dx.doi.org/10.1016/j.rdc.2014.09.003] [PMID: 25399936]

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