Exacerbação aguda da fibrose pulmonar idiopática

 

Natália Melo 1

Carla Damas 2

Conceição Souto Moura 3

António Morais 2

 

 

Resumo

Alguns doentes com fibrose pulmonar idiopática (FPI) apresentam durante a sua evolução fases deagravamento clínico sem causa conhecida, designadas como “exacerbação aguda” ou “fase acelerada” da doença (EA). Caracterizam -se pelo agravamento marcado da dispneia, hipoxemia e pelo aparecimento de novas opacidades pulmonares ou pelo agravamento das já existentes no estudo imagiológico. Os achados histológicos típicos são o dano alveolar difuso (DAD) sobreposto a alterações de pneumonia intersticial usual (UIP). Esta entidade clínica associa -se a uma mortalidade elevada, não havendo até ao momento nenhuma terapêutica de comprovada eficácia.

Os autores descrevem os casos clínicos de cinco doentes que apresentaram alterações clínicas, funcionais e radiológicas sugestivas de EA-FPI, assim como o tratamento efectuado e a evolução observada, enquadrando-os na discussão das características normalmente apresentadas por esta entidade.

Palavras -chave: Fibrose pulmonar idiopática, exacerbação aguda.

 

 

Acute exacerbation of idiopathic pulmonary fibrosis

Abstract

Some patients with Idiopathic Pulmonary Fibrosis (IPF) have disease accelerated deterioration without identifiable cause referred as “acute exacerbation” or “accelerated stage”. It is characterized by severe worsening of dyspnea, hypoxemia and new or progressive opacities on imaging studies. The typical histological findings are diffuse alveolar damage in addition to the features of usual interstitial pneumonia pattern. Mortality in this clinical entity is very high and no efficacious therapeutic have been described.

The authors describe the clinical, functional and radiological features, treatment and evolution of five patients with IPF acute exacerbation. A discussion will be carry out concerning the IPF acute exacerbation usual features comparing with the alterations noticed in those patients.

Key words: Idiopathic pulmonary fibrosis, acute exacerbation.

 

 

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Bibliografia

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1 Interna Complementar de Pneumologia

2 Assistente Hospitalar de Pneumologia

Serviço de Pneumologia do Hospital S. João – Director de Serviço: Prof. Dr. Venceslau Hespanhol

3 Assistente Hospitalar Graduada de Anatomia Patológica

Serviço de Anatomia Patológica do Hospital S. João – Directora de Serviço: Prof. Dra. Fátima Carneiro

 

Correspondência/Correspondence to:

Natália Melo

Serviço de Pneumologia, Hospital de S. João

Alameda Professor Hernâni Monteiro

4202-451 Porto

Telefone: 919371855

e -mail: nataliafmelo@hotmail.com

 

Recebido para publicação/received for publication: 08.04.11

Aceite para publicação/accepted for publication: 08.11.07