João Bento1
Gabriela Fernandes2
Maria Alice Barbosa3
Adriana Magalhães3
Ana Rosa Santos4
Venceslau Hespanhol4
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.
Palavras-chave: Esclerodermia, cancro, pulmão, fibrose.
Lung adenocarcinoma associated with systemic sclerosis: A case report
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.
Key-words: Systemic sclerosis, cancer, lung, fibrosis.
Texto completo disponível apenas em PDF.
Full text only available in PDF format.
Bibliografia
1. Chifflot H, Fautrel B, Sordet C, Chatelus E, Sibilia J. Incidence and prevalence of systemic sclerosis: a systematic literature review. Semin Arthritis Rheum 2008; 37(4): 223-35. [ Links ]
2. Hesselstrand R, Scheja A, Akesson A. Mortality and causes of death in a Swedish séries of systemic sclerosis patients. Ann Rheum Dis 1998; 57:682-6.
3. Witt Christian, Borges Adrian C, John Mathias, Fietze Ingo, Baumann Gert, Krause Andreas. Pulmonary involvement in diffuse cutaneous systemic sclerosis: broncheoalveolar fluid granulocytosis predicts progreesion of fibrosing alveolitis. Ann Rheum Dis 1999; 58: 635-40.
4. Pearson J E, Silman A J. Risk of cancer in patients with scleroderma. Ann Rheum Dis 2003; 62:697-9.
5. Hill CL, Nguyen A-M, Roder D, Roberts–Thomson P. Risk of cancer in patients with scleroderma: a population based cohort study. Ann Rheum Dis 2003; 62:728-31.
6. Kanaji N, Fujita J, Bandoh S, Fukumoto T, Ishikawa M, Haba R, Ishii T, Kubo A, Ishida T. Small cell lung cancer associated with systemic sclerosis. Internal Medicine 2005; 44:315-8.
7. Bouros D, Hatzakis K, Labrakis H, Zeibecoglou K. Association of malignancy with diseases causing interstitial pulmonary changes. Chest 2002; 121:1278-89.
8. Zatuchni J, Campbell WN, Zarafonetis CJD. Pulmonary fibrosis and terminal bronchiolar (“alveolar-cell”. carcinoma in scleroderma. Cancer 1953; 6:1147-58.
9. Bernatsky S, Ramsey-Goldman R, Clarke A. Malignancy and autoimmunity. Curr Opin Rheumatol 2006; 18(2):129-34.
10. Abu-Shakra M, Guillemin F, Lee P. Cancer in systemic sclerosis. Arthritis Rheum 1993; 36:460-4.
11. Rosenthal AK, McLaughlin JK, Linet MS, Persson I. Scleroderma and malignancy: an epidemiological study. Ann Rheum Dis 1993; 52:531-3.
]]> 12. Rosenthal A K, McLaughlin J K, Gridley G, Nyrén O. Incidence of cancer among patients with systemic sclerosis. Cancer 1995; 76:910-4.13. Winkelmann RK, Flach DB, Unni KK. Lung cancer and scleroderma. Arch Dermatol Res 1988; 280 (Suppl): S15-18.
1 Interno Complementar de Pneumologia
2 Assistente Hospitalar de Pneumologia
3 Assistente Hospitalar Graduado de Pneumologia
4 Chefe de Serviço de Pneumologia
Serviço de Pneumologia, Hospital de São João, EPE, Porto
]]> Recebido para publicação/received for publication: 08.04.21
Aceite para publicação/accepted for publication: 08.09.12
]]>