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Pulmonary Amyloidosis: A Diagnostic Challenge

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Main Author: Alves, Ana
Publication Date: 2015
Other Authors: Alfaro, Tiago M., Madama, Daniela, Freitas, Sara, Robalo-Cordeiro, Carlos, Gamboa, Fernanda
Format: Article
Language: por
Source: Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
Download full: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/5948
Summary: Amyloidosis is characterized by amyloid extracellular deposition in organs and tissues. Pulmonary involvement is a rare manifestation of the disease and it can be focal or as part of systemic amyloidosis. We report two cases. Case 1: 71 year-old female with bronchiectasis and Sjogren’s syndrome, who complained of anorexia, weight loss and a productive cough. The diagnostic study included a surgical lung biopsy and histological examination demonstrated pulmonary amyloidosis. Case 2: 83 year-old male patient, ex-smoker, asymptomatic,whose routine chest x-ray showed a nodular opacity in the right lung field. A transthoracic biopsy revealed an amyloid lung tumor. These cases illustrate a rare disease which in Case 1 also coexisted with Sjögren’s syndrome and bronchiectasis. The most important differential diagnosis is cancer and so a definitive diagnosis is essential, as amyloidosis is usually benign and indolent.
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spelling Pulmonary Amyloidosis: A Diagnostic ChallengeAmiloidose Pulmonar: Um Desafio DiagnósticoAmyloidosisBronchiectasisLung DiseasesSjögren’s Syndrome.AmiloidoseBronquiectasiasDoenças do PulmãoSíndrome de Sjögren.Amyloidosis is characterized by amyloid extracellular deposition in organs and tissues. Pulmonary involvement is a rare manifestation of the disease and it can be focal or as part of systemic amyloidosis. We report two cases. Case 1: 71 year-old female with bronchiectasis and Sjogren’s syndrome, who complained of anorexia, weight loss and a productive cough. The diagnostic study included a surgical lung biopsy and histological examination demonstrated pulmonary amyloidosis. Case 2: 83 year-old male patient, ex-smoker, asymptomatic,whose routine chest x-ray showed a nodular opacity in the right lung field. A transthoracic biopsy revealed an amyloid lung tumor. These cases illustrate a rare disease which in Case 1 also coexisted with Sjögren’s syndrome and bronchiectasis. The most important differential diagnosis is cancer and so a definitive diagnosis is essential, as amyloidosis is usually benign and indolent.A amiloidose caracteriza-se pela deposição extracelular de substância amilóide em diferentes órgãos e tecidos. O atingimento pulmonar é raro e pode acontecer de forma isolada ou associado a doença sistémica. Descrevem-se dois casos clínicos. Caso 1: Mulher de 71 anos, com antecedentes de bronquiectasias e síndrome de Sjögren, que referia anorexia, emagrecimento e tosse produtiva. O estudo incluiu uma biópsia pulmonar cirúrgica que demonstrou amiloidose localizada ao aparelho respiratório. Caso 2: Homem de 83anos, ex-fumador, assintomático. Enviado por opacidade nodular em radiografia de tórax de rotina. A biópsia pulmonar transtorácica revelou um tumor amilóide do pulmão (amiloidoma). Estes casos ilustram uma doença rara que no Caso 1 está associada a bronquiectasias e síndrome de Sjögren. O diagnóstico diferencial mais importante é a patologia neoplásica, sendo o diagnóstico definitivo importante, já que a amiloidose pulmonar é geralmente benigna e indolente.Ordem dos Médicos2015-07-10info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegapplication/mswordhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/5948oai:ojs.www.actamedicaportuguesa.com:article/5948Acta Médica Portuguesa; Vol. 28 No. 4 (2015): July-August; 530-533Acta Médica Portuguesa; Vol. 28 N.º 4 (2015): Julho-Agosto; 530-5331646-07580870-399Xreponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiainstacron:RCAAPporhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/5948https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/5948/4431https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/5948/7431https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/5948/7432https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/5948/7433https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/5948/7434https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/5948/7438https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/5948/7578Alves, AnaAlfaro, Tiago M.Madama, DanielaFreitas, SaraRobalo-Cordeiro, CarlosGamboa, Fernandainfo:eu-repo/semantics/openAccess2022-12-20T11:04:39Zoai:ojs.www.actamedicaportuguesa.com:article/5948Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireinfo@rcaap.ptopendoar:https://opendoar.ac.uk/repository/71602025-05-28T10:40:12.214442Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiafalse
dc.title.none.fl_str_mv Pulmonary Amyloidosis: A Diagnostic Challenge
Amiloidose Pulmonar: Um Desafio Diagnóstico
title Pulmonary Amyloidosis: A Diagnostic Challenge
spellingShingle Pulmonary Amyloidosis: A Diagnostic Challenge
Alves, Ana
Amyloidosis
Bronchiectasis
Lung Diseases
Sjögren’s Syndrome.
Amiloidose
Bronquiectasias
Doenças do Pulmão
Síndrome de Sjögren.
title_short Pulmonary Amyloidosis: A Diagnostic Challenge
title_full Pulmonary Amyloidosis: A Diagnostic Challenge
title_fullStr Pulmonary Amyloidosis: A Diagnostic Challenge
title_full_unstemmed Pulmonary Amyloidosis: A Diagnostic Challenge
title_sort Pulmonary Amyloidosis: A Diagnostic Challenge
author Alves, Ana
author_facet Alves, Ana
Alfaro, Tiago M.
Madama, Daniela
Freitas, Sara
Robalo-Cordeiro, Carlos
Gamboa, Fernanda
author_role author
author2 Alfaro, Tiago M.
Madama, Daniela
Freitas, Sara
Robalo-Cordeiro, Carlos
Gamboa, Fernanda
author2_role author
author
author
author
author
dc.contributor.author.fl_str_mv Alves, Ana
Alfaro, Tiago M.
Madama, Daniela
Freitas, Sara
Robalo-Cordeiro, Carlos
Gamboa, Fernanda
dc.subject.por.fl_str_mv Amyloidosis
Bronchiectasis
Lung Diseases
Sjögren’s Syndrome.
Amiloidose
Bronquiectasias
Doenças do Pulmão
Síndrome de Sjögren.
topic Amyloidosis
Bronchiectasis
Lung Diseases
Sjögren’s Syndrome.
Amiloidose
Bronquiectasias
Doenças do Pulmão
Síndrome de Sjögren.
description Amyloidosis is characterized by amyloid extracellular deposition in organs and tissues. Pulmonary involvement is a rare manifestation of the disease and it can be focal or as part of systemic amyloidosis. We report two cases. Case 1: 71 year-old female with bronchiectasis and Sjogren’s syndrome, who complained of anorexia, weight loss and a productive cough. The diagnostic study included a surgical lung biopsy and histological examination demonstrated pulmonary amyloidosis. Case 2: 83 year-old male patient, ex-smoker, asymptomatic,whose routine chest x-ray showed a nodular opacity in the right lung field. A transthoracic biopsy revealed an amyloid lung tumor. These cases illustrate a rare disease which in Case 1 also coexisted with Sjögren’s syndrome and bronchiectasis. The most important differential diagnosis is cancer and so a definitive diagnosis is essential, as amyloidosis is usually benign and indolent.
publishDate 2015
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dc.publisher.none.fl_str_mv Ordem dos Médicos
publisher.none.fl_str_mv Ordem dos Médicos
dc.source.none.fl_str_mv Acta Médica Portuguesa; Vol. 28 No. 4 (2015): July-August; 530-533
Acta Médica Portuguesa; Vol. 28 N.º 4 (2015): Julho-Agosto; 530-533
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