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Vol. 15. Issue 5.
Pages 929-935 (September - October 2009)
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Vol. 15. Issue 5.
Pages 929-935 (September - October 2009)
Caso Clínico/Case Report
Open Access
Granulomatose de Wegener – Envolvimento otológico, nasal, laringotraqueal e pulmonar
Wegener granulomatosis – Otologic, nasal, tracheobronchial and pulmonary involvement
Visits
5853
Sandra Figueiredo1,
Corresponding author
sandrabugalho@iol.pt

Hospital de São João (EPE), Porto.
, Laurentino Mendes Leal2, António Morais3, Adriana Magalhães4, Teresa Oliveira5, Venceslau Hespanhol4, Carlos Dias6, Gabriela Fernandes3
1 Internato Médico. Serviço de Pneumologia)
2 Internato Médico. Serviço de Otorrinolaringologia
3 Assistente Hospitalar. Serviço de Pneumologia
4 Assistente Hospitalar Graduado. Serviço de Pneumologia
5 Assistente Hospitalar Graduada. Unidade de Cuidados Intensivos Polivalentes
6 Assistente Hospitalar Graduado. Serviço de Medicina Interna
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Resumo

A granulomatose de Wegener é uma vasculite sistémica rara e idiopática caracterizada pelo atingimento dos pequenos vasos. A doença atinge, preferencial-mente, as vias aérea superior e inferior e os rins, levando à formação de granulomas e necrose destes órgãos. As manifestações clínicas e o envolvimento orgânico variam largamente. O diagnóstico e tratamento precoce podem levar à recuperação total. No entanto, o atraso no diagnóstico pode ser fatal. Os autores apresentam o caso de uma doente de 33 anos com uma forma de apresentação grave e rara da doença, mas com uma evolução favorável após diagnóstico e tratamento adequado.

Rev Port Pneumol 2009; XV (5): 929-935

Palavras-chave:
Granulomatose de Wegener
forma limitada grave
Abstract

Wegener granulomatosis is a rare systemic idiopathic disease characterized by involvement of small vessels – medium and small arteries, venules, arterioles and ocasionally large arteries. This disease has predilection for the upper and lower respiratory tract and the kidney, with granulomatous inflamation and necrosis. Clinical manifestations and organ involvement of the disease vary widely. Early diagnosis and treatment may lead to a full recovery. Without treatment, Wegener’s granulomatosis can be fatal. The authors present a case of a 33 year-old female, with severe disease, but with good outcome, after adequate diagnosis and treatment.

Rev Port Pneumol 2009; XV (5): 929-935

Key-words:
Wegener´s granulomatosis
severe limited form
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Copyright © 2009. Sociedade Portuguesa de Pneumologia/SPP
Pulmonology
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