Epistaxis como primeira manifestação de granulomatose Wegener. Caso clínico

Autores

  • A. Casas Novas Interna Complementar de Otorrinolaringologia, do Centro Hospitalar Lisboa Central, EPE, Hospital Dona Estefânia
  • I. Moreira Interna Complementar de Otorrinolaringologia, do Centro Hospitalar Lisboa Central, EPE, Hospital Dona Estefânia
  • P. Brazão Santos Assistente Hospitalar de Otorrinolaringologia, do Centro Hospitalar Lisboa Central, EPE, Hospital Dona Estefânia
  • L. Monteiro Directora de Serviço de Otorrinolaringologia do Centro Hospitalar Lisboa Central, EPE, Hospital Dona Estefânia

DOI:

https://doi.org/10.34631/sporl.271

Palavras-chave:

epistaxis, Granulomatose Wegener, vasculite, granuloma

Resumo

A Granulomatose de Wegener é uma doença sistémica de etiologia desconhecida, caracterizada pela tríade clínica: envolvimento da mucosa nasal e dos seios perinasais, infiltração e cavitação pulmonar e doença renal com hematúria. A Granulomatose de Wegener afecta normalmente adultos jovens e de meia idade e embora seja incomum nas crianças, pode surgir em qualquer estrato etário.

Apresentamos o caso clínico de uma adolescente de 14 anos, com sintomas de epistaxis, cuja investigação posterior mostrou insuficiência renal e múltiplos nódulos pulmonares. O teste positivo para o anticorpo antineutrofílico citoplasmático suportou o diagnóstico de Granulomatose de Wegener, necessitando de terapêutica com hemodiálise e imunossupressão.

Referências

Akikusa JD, Schneider R, Harvey EA, Hebert D, Thorner PS, Laxer RM, Silverman ED.Clinical features and outcome of pediatric Wegener's granulomatosis. Arthritis Rheum. 2007 May 25;57(5):837-844

Alata F, Metinta M, Ozkan R, Erginel S, Uçgun I, Yildirim H. Wegener granulomatosis: three cases. Tuberk Toraks. 2003;51{4):440-5.

Ayala de la Cruz Mdel C, González Díaz R, López Lara ND. Wegener's Granulomatosis. Report of pediatric case and review of the literature, Ver Aler Mex. 2003 Mar-Apr; 50{2):71-5.

Gottschlich S, Ambrosch P, Kramkowski D, Laudien M, Buchelt T, Gross WL, Hellmich B. Head and neck manifestations of Wegener's granulomatosis Rhinology. 2006 Dec;44(4):227-33

Neil Tanna MD. Wegener Granulomatosis, eMedicine from Web. 1-23,October 2006

Gubbels SP, Barkhuizen A, Hwang PH. Head and Neck manifestations of Wegener's Granulomatosis. Otolaryngol Clin N Am 2003;685-705

James P. Orlowski M.D., John D. Clough M.D., and Paul G. Dyment M.D. Wegener's Granulomatosis in the Pediatric Age Group.Pediatrics Vol 61 No 1January 1978, pp 83-90

R. Erickson V,H Hwang P. Wegener's granulomatosis: current trends in diagnosis and management. Cur Opin Otolaryngol Head Neck Surg 2007, 15:1170-176

Metaxaris G, Prokopakis EP, Karatzanis AD, Sakelaris G, Heras P, Velegrakis GA, Helidonis ES. Otolaryngologic manifestations of small vessel vasculitis. Auris Nasus Larynx. 2002 Oct;29(4):353-6

Levine D, Akikusa J, Manson D, Silverman E, Schneider R. Chest CT findings in pediatric Wegener's granulomatosis. Pediatr Radiol. 2007 Jan;37(1):57-62

Passey J, Walker R. Wegener's granulomatosis: an unusual cause of upper airway obstruction. Case Report Anaesthesia. 55(7):682-684, July 2000

Dipika A, Balakrishnan C, Nene A, Santosh G, Udwadia ZF. Upper airways involvement in Wegener's Granulomatosis. A case report and review, J Indian Rheumatol. Assoc. 2004: 12: 0-0

Hall SL, Miller LC, Duggan E, Mauer SM, Beatty EC, Hellerstein S. Wegener granulomatosis in pediatric patients. J Pediatr. 1985 May; 106(5):739-44.

Hewins P, Tervaert JW, Savage CO, Kallenberg CG. Is Wegener's granulomatosis an autoimmune disease? Curr Opin Rheumatol 2000; 12: 3-10.

Hughes RG, Drake-Lee A. Nasal manifestations of granulomatous disease. Hosp Med. 2001 Jul;62(7):417-21

Khaled Shafiei, MD, Euil Luther, MD, Michael Archie, MD, Janis Gulick, MD and Marjorie R. Fowler, MD. Wegener Granulomatosis: Case Report and Brief Literature Review. The Journal of the American Board of Family Practice 16:555-559 (2003)

Llompart X, Aumaí'tre O, Kémény JL, Mom T, Gilain L. Early otorhinolaryngological manifestations of Wegener's granulomatosis. Analysis of 21 patients. Ann Otolaryngol Chir Cervicofac. 2002 Dec;119(6):330-6

Skálová S, Minxová L, Podhola M Wegener's granulomatosis in a 15-year-old boy Turk J Pediatr. 2003 Oct-Dec;45(4):353-6.

Snow J, Ballenger MD, Otorhinolaryngology Head and Neck Surgery, 6th edition, BC Decker Inc, 2003; 755-756

Spronk MH, Pals ST, Haumann TJ, Schouten-van Meeteren AY. A child with Wegener's granulomatosis Tijdschr Kindergeneeskd. 1992 Apr;60(2):52-6.

Thapar A, George A, Pfleiderer A. Fulminant Wegener's granulomatosis presenting as epistaxis. Emerg Med J. 2007 Mar;24 {3) e14.

Vignes S, Chaillet M, Cabane J, Piette JC. Nasal septal perforation and systemic disease. Rev Med Interne. 2002 Nov;23(11):919-26

Von Scheven E, Lee C, Berg BO. Pediatric Wegener's granulomatosis complicated by central nervous system vasculitis. Pediatr Neurol. 1998 Oct;19(4):317

Yalcindag A, Sundel R. Vasculitis in childhood. Curr Opin Rheumatol. 2001 Sep;13(5):422-7.

Downloads

Como Citar

Casas Novas, A., Moreira, I., Brazão Santos, P., & Monteiro, L. (2010). Epistaxis como primeira manifestação de granulomatose Wegener. Caso clínico. Revista Portuguesa De Otorrinolaringologia-Cirurgia De Cabeça E Pescoço, 48(2), 89–92. https://doi.org/10.34631/sporl.271

Edição

Secção

Caso Clínico