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Abordagem do doente com fibrose pulmonar idiopática
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A fibrose pulmonar idiopática (IPF) é uma doença difusa do interstício pulmonar (DDIP) que se manifesta por fibrose progressiva e irreversível e que afeta principalmente adultos de idade avançada. Apesar dos avanços crescentes na abordagem terapêutica, o prognóstico da IPF é mau e a doença tende a evoluir inevitavelmente para falência respiratória terminal ou para outras complicações como o cancro do pulmão, com sobrevida média entre os 2 a 4 anos após o diagnóstico.
Trata-se de uma patologia com etiologia desconhecida embora de provável relação com a interação entre alterações genéticas que afetam a integridade das células epiteliais alveolares, fatores ambientais e alterações que levam ao envelhecimento precoce das células.
Estes doentes apresentam-se geralmente com quadros sintomáticos inespecíficos caracterizados por dispneia de esforço progressiva e tosse não produtiva. Pela baixa especificidade dos sintomas, a tomografia computorizada de alta resolução (TCAR) torna-se o método de diagnóstico central da IPF, sendo que o padrão imagiológico que melhor define esta doença é o padrão UIP (usual interstitial pneumonia). Segundo as orientações mais recentes da American Thoracic Society, European Respiratory Society, Japanese Respiratory Society e Latin American Thoracic Society (ATS/ERS/JRS/ALAT), um diagnóstico preciso requer primariamente a exclusão de outras DDIP de causa conhecida concomitantemente com a presença do padrão de UIP na TCAR ou de combinações específicas dos padrões imagiológicos encontrados na TCAR com os padrões histopatológicos em doentes submetidos a biópsia pulmonar. Em casos inconclusivos, a abordagem multidisciplinar torna-se preponderante na obtenção de um diagnóstico definitivo. Na marcha diagnóstica podem ainda ser necessários outros exames complementares (lavado broncoalveolar, serologias, testes de função respiratória) na tentativa de exclusão de outros diagnósticos ou na avaliação do prognóstico e evolução da doença.
A Pirfenidona e o Nintedanib são, até o momento, os únicos fármacos aprovados e recomendados que demonstraram eficácia confirmada na redução do declínio funcional e na progressão da doença. Nos casos de doença terminal, o transplante pulmonar constitui a única terapêutica disponível capaz de melhorar a sobrevida e restaurar a função pulmonar. O algoritmo do tratamento deve ainda incluir opções não farmacológicas, como a oxigenoterapia em doentes com hipoxemia, a reabilitação respiratória e os cuidados paliativos, que apesar de não alterarem o curso da doença, conferem melhor qualidade de vida aos doentes. Inclui ainda o tratamento de comorbilidades e complicações frequentes que interferem com o prognóstico da doença, como é o caso do enfisema pulmonar, das exacerbações agudas, da doença do refluxo gastroesofágico e a síndrome da apneia obstrutiva do sono.
Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease that mostly affects people of advanced age and is characterized by progressive and irreversible fibrosis. Despite the ever-changing updates to the therapeutic approach of IPF, its prognosis is still poor and the disease tends to inevitably evolve into terminal respiratory failure or other complications, such as lung cancer. Accordingly, the median survival time from the date of diagnosis is 2 to 4 years. Though the etiology of IPF is still unknown, it is hypothesized that the disease develops as a result of an interaction between a genetic architecture affecting epithelial cell integrity, environmental factors and accelerated ageing-associated changes. The clinical symptoms of IPF are usually nonspecific, but generally include exertional dyspnea and a non-productive cough. The low specificity of the clinical presentation gave rise to the central role that high resolution CT (HRCT) plays on the diagnosis of this pathology nowadays, with the most typical finding being the usual interstitial pneumonia (UIP) pattern. According to the most recent guidelines by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society and Latin American Thoracic Society (ATS/ERS/JRS/ALAT), the diagnosis of IPF requires the exclusion of other known causes of ILD and either the presence of the HRCT patterns of UIP or specific combinations of HRCT patterns and histopathology patterns in patients subjected to lung biopsy. In cases that remain dubious, a multidisciplinary approach is preponderant in the establishment of the definitive diagnosis. The diagnostic workup might entail other tests, such as a bronchoalveolar lavage, serological screening and pulmonary function testing, so as to rule out other diagnostic hypotheses or allow a prognostic and disease progression evaluation. So far, Pirfenidone and Nintedanib are the only drugs to have shown efficacy in slowing functional decline and disease progression, granting their approval and recommendation for the treatment of IPF. When it comes to advanced stage disease, lung transplantation seems to be the only therapeutic option associated with improved survival rates and restored pulmonary function. The treatment algorithm should also comprise non-pharmacological options, such as oxygen therapy in patients with hypoxemia, pulmonary rehabilitation and palliative care, as these measures, even if not changing the clinical course of the disease, have demonstrated a beneficial effect on quality of life. The treatment of common comorbidities and complications, such as pulmonary emphysema, acute exacerbations, gastro-oesophageal reflux and obstructive sleep apnoea, should also be contemplated, as these affect the long-term prognosis.
Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease that mostly affects people of advanced age and is characterized by progressive and irreversible fibrosis. Despite the ever-changing updates to the therapeutic approach of IPF, its prognosis is still poor and the disease tends to inevitably evolve into terminal respiratory failure or other complications, such as lung cancer. Accordingly, the median survival time from the date of diagnosis is 2 to 4 years. Though the etiology of IPF is still unknown, it is hypothesized that the disease develops as a result of an interaction between a genetic architecture affecting epithelial cell integrity, environmental factors and accelerated ageing-associated changes. The clinical symptoms of IPF are usually nonspecific, but generally include exertional dyspnea and a non-productive cough. The low specificity of the clinical presentation gave rise to the central role that high resolution CT (HRCT) plays on the diagnosis of this pathology nowadays, with the most typical finding being the usual interstitial pneumonia (UIP) pattern. According to the most recent guidelines by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society and Latin American Thoracic Society (ATS/ERS/JRS/ALAT), the diagnosis of IPF requires the exclusion of other known causes of ILD and either the presence of the HRCT patterns of UIP or specific combinations of HRCT patterns and histopathology patterns in patients subjected to lung biopsy. In cases that remain dubious, a multidisciplinary approach is preponderant in the establishment of the definitive diagnosis. The diagnostic workup might entail other tests, such as a bronchoalveolar lavage, serological screening and pulmonary function testing, so as to rule out other diagnostic hypotheses or allow a prognostic and disease progression evaluation. So far, Pirfenidone and Nintedanib are the only drugs to have shown efficacy in slowing functional decline and disease progression, granting their approval and recommendation for the treatment of IPF. When it comes to advanced stage disease, lung transplantation seems to be the only therapeutic option associated with improved survival rates and restored pulmonary function. The treatment algorithm should also comprise non-pharmacological options, such as oxygen therapy in patients with hypoxemia, pulmonary rehabilitation and palliative care, as these measures, even if not changing the clinical course of the disease, have demonstrated a beneficial effect on quality of life. The treatment of common comorbidities and complications, such as pulmonary emphysema, acute exacerbations, gastro-oesophageal reflux and obstructive sleep apnoea, should also be contemplated, as these affect the long-term prognosis.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2019
Palavras-chave
Doenças difusas do interstício pulmonar Fibrose pulmonar idiopática Pneumologia