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Vol. 15. Issue 1.
Pages 93-99 (January - February 2009)
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Vol. 15. Issue 1.
Pages 93-99 (January - February 2009)
Caso Clínico/Case Report
Open Access
Adenocarcinoma do pulmão em doente com esclerodermia: Um caso clínico
Lung adenocarcinoma associated with systemic sclerosis: A case report
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João Bento1, Gabriela Fernandes2, Maria Alice Barbosa3, Adriana Magalhães3, Ana Rosa Santos3, Venceslau Hespanhol4
1 Interno Complementar de Pneumologia, Serviço de Pneumologia, Hospital de São João, EPE, Porto.
2 Assistente Hospitalar de Pneumologia, Serviço de Pneumologia, Hospital de São João, EPE, Porto.
3 Assistente Hospitalar Graduado de Pneumologia, Serviço de Pneumologia, Hospital de São João, EPE, Porto.
4 Chefe de Serviço de Pneumologia, Serviço de Pneumologia, Hospital de São João, EPE, Porto.
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Resumo

A esclerodermia é uma doença do tecido conjuntivo de etiologia desconhecida, que tem sido associada a um risco acrescido de malignidade. O cancro do pulmão é a neoplasia mais frequente, nestes doentes.

Apresenta-se o caso clínico de uma mulher de 42 anos, não fumadora, com esclerodermia, que desenvolveu agravamento progressivo do seu estado geral e derrame pleural com características de exsudado, sem evidência de infecção ou malignidade. A TAC torácica mostrou zonas de fibrose, a broncofibroscopia, os lavados brônquico e broncoalveolar foram normais. Foi excluída neoplasia extrapulmonar. Na pleuroscopia, observaram-se formações nodulares, cujas biópsias revelaram tratar-se de adenocarcinoma pulmonar. Iniciou quimioterapia, desenvolvendo ao 48.º dia neutropenia febril e sépsis, vindo a morrer 12 dias depois.

Salientamos este caso pela relação, apesar de rara, entre a esclerodermia e o cancro do pulmão e pela importância de uma vigilância pulmonar cuidadosa, em indivíduos com esta doença reumatológica, pelo risco acrescido de neoplasia.

Rev Port Pneumol 2009; XV (1): 93-99

Palavras-chave:
Esclerodermia
cancro
pulmão
fibrose
Abstract

Systemic sclerosis (scleroderma) is a connective tissue disorder of unknown aetiology characterised by immune abnormalities, which has been related to an increased risk of malignancy. Lung cancer is the most prevalent among these patients.

We present a clinical case of a 42 years old non smoker female with systemic sclerosis. She presented progressive general health status worsening and an exudative pleural effusion, with no evidence of infection or malignancy. Chest high resolution computed tomography revealed pulmonary fibrosis. Bronchoscopy, bronchial and bronchoalveolar lavage were normal. Extra-pulmonary malignancies were excluded. Pleural nodularities were found on pleuroscopy and the biopsy was compatible with lung adenocarcinoma. Chemotherapy was then started, which complicated with febrile neutropenia, sepsis and patient death 12 days after.

The purpose of this case report is to bring attention to the relationship, although rare, between systemic sclerosis and lung cancer, stressing the importance of adequate pulmonary surveillance in these patients.

Rev Port Pneumol 2009; XV (1): 93-99

Key-words:
Systemic sclerosis
cancer
lung
fibrosis
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Copyright © 2009. Sociedade Portuguesa de Pneumologia/SPP
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