Journal of Medical Case Reports | |
Granulomatosis with polyangiitis (Wegener’s) as a necrotizing gingivitis mimic: a case report | |
Jesse Pewarchuk1  Katrina Genuis1  | |
[1] Island Medical Program, UBC Faculty of Medicine, Medical Science Building, University of Victoria, PO Box 1700 STN CSC, Victoria, BC V8W 2Y2, Canada | |
关键词: Rituximab; ANCA; Necrotizing gingivitis; Wegener’s; Granulomatosis with polyangiitis; | |
Others : 1181055 DOI : 10.1186/1752-1947-8-297 |
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received in 2014-03-14, accepted in 2014-07-12, 发布年份 2014 | |
【 摘 要 】
Introduction
Granulomatosis with polyangiitis poses a significant diagnostic dilemma due its diverse presentations. Seemly isolated sites of disease, such as oral ulcers, may present to physicians working in primary care settings, the emergency room, and subspecialty fields as well as to dentists. Oral presentations are particularly challenging to identify and require a high index of suspicion and a detailed knowledge of the condition in order to diagnose and treat. We detail a case of granulomatosis with polyangiitis presenting as necrotizing gingivitis, one of the first of its kind to be reported.
Case presentation
An otherwise healthy 32-year-old, Caucasian woman presented to various physicians with progressive, painful oral ulcers. Following consultations with multiple primary care physicians and subspecialties, an initial diagnosis of severe infectious necrotizing gingivitis was made resulting in combination antibiotic treatment as well as surgical debridement involving extraction of all maxillary and three mandibular teeth. With the discovery of a positive cytoplasmic anti-neutrophil cytoplasmic antibody and a constellation of associated systemic symptoms, our patient was subsequently diagnosed with granulomatosis with polyangiitis. The treatment regimen of rituximab and methylprednisone was chosen in consideration of our patient’s desire for future fertility and has been successful in inducing and maintaining remission.
Conclusions
Following the case presentation, we review the current literature regarding granulomatosis with polyangiitis presentation, diagnosis and treatment. In discussing features of granulomatosis with polyangiitis presentation, diagnostic tests, and important new treatment options, we seek to enable physicians of all specialties to better recognize and begin appropriate treatment for this complex condition.
【 授权许可】
2014 Genuis and Pewarchuk; licensee BioMed Central Ltd.
【 预 览 】
Files | Size | Format | View |
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20150514105729667.pdf | 404KB | download | |
Figure 1. | 89KB | Image | download |
【 图 表 】
Figure 1.
【 参考文献 】
- [1]Hoffman GS, Langford CA, Specks U: Granulomatosis with polyangiitis (Wegener’s). In Inflammatory Diseases of Blood Vessels. Second edition edition. Edited by Hoffman GS, Weyand CM, Langford CA, Goronzy JJ. Chichester, West Sussex: Wiley-Blackwell; 2012:238-251.
- [2]Langford CA: Clinical features and diagnosis of small-vessel vasculitis. Cleve Clin J Med 2012, Suppl 3:S3-S7.
- [3]Stone JH: Granulomatosis with polyangiitis (Wegener granulomatosis). In Current Rheumatology Diagnosis and Treatment. Edited by Imboden JB, Hellmann DB, Stone J. New York: Lange Medical Books/McGraw-Hill, Medical Publishing Division; 2007. Available: http://www.accessmedicine.com.ezproxy.library.ubc.ca/content.aspx?aID=57273181 webcite (accessed 2013 August 20)
- [4]Langford CA, Hoffman GS: Rare diseases.3: Wegener's granulomatosis. Thorax 1999, 54:629-637.
- [5]Patten SF, Tomecki KJ: Wegener’s granulomatosis: cutaneous and oral mucosal disease. J Am Acad Dermatol 1993, 28:710-718.
- [6]Manchanda YM, Tejasvi T, Handa R, Ramam M: Strawberry gingiva: a distinctive sign in Wegener’s granulomatosis. J Am Acad Dermatol 2003, 49:335-337.
- [7]McGregor JG, Hogan SL, Hu Y, Jennette CE, Falk RJ, Nachman PH: Glucocorticoids and relapse and infection rates in anti-neutrophil cytoplasmic antibody disease. Clin J Am Soc Nephrol 2012, 7:240-247.
- [8]Rutgers A, Sanders JSF, Tervaert JWC, Kallenberg CGM: Autoantibodies and vascular inflammation. In Inflammatory Diseases of Blood Vessels. Second edition edition. Edited by Hoffman GS, Weyand CM, Langford CA, Goronzy JJ. Chichester, West Sussex: Wiley-Blackwell; 2012:61-70.
- [9]De Groot K, Harper L, Jayne DR, Flores Suarez LF, Gregorini G, Gross WL, Luqmani R, Pusey CD, Rasmussen N, Sinico RA, Tesar V, Vanhille P, Westman K, Savage CO: Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med 2009, 150:670-680.
- [10]Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, Kallenberg CG, St Clair EW, Turkiewicz A, Tchao NK, Webber L, Ding L, Sejismundo LP, Mieras K, Weitzenkamp D, Ikle D, Seyfert-Margolis V, Mueller M, Brunetta P, Allen NB, Fervenza FC, Geetha D, Keogh KA, Kissin EY, Monach PA, Peikert T, Stegeman C, Ytterberg SR, Specks U: Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 2010, 363:221-232.