Fibrose pulmonar idiopática: uma década de progressos

Swigris, Jeffrey J.; Brown, Kevin K.

doi:10.1590/s1806-37132006000300012

Cited by 3 publications

(1 citation statement)

References 54 publications

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“…Esse é o aspecto chave no processo de diagnóstico. Anos atrás o tratamento da doença ainda se constituía como uma incógnita, pois não havia fármacos específicos para tratá-la, sendo assim, eram utilizadas diversas classes terapêuticas, especialmente os glicocorticoides, que foram amplamente utilizados, porém não resultavam em regressão da condição clínica dos pacientes (Swigris & Brown, 2006 ).…”

Section: Introductionunclassified

Itinerário de utentes com fibrose pulmonar idiopática atendidos via judicial ou administrativa pela Secretaria de Estado de Saúde do Pará

Kaminosono

Gillet

Sousa

et al. 2021

RSD

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Objetivo: Investigar o itinerário de utentes que tiveram acesso aos medicamentos antifibróticos nintedanibe e pirfenidona via judicial ou administrativa; assim como, seus respectivos perfis socioeconômicos e epidemiológicos atendidos na Farmácia de Demandas Judiciais e Administrativas. Método: Trata-se de estudo transversal, retrospectivo, analítico-descritivo, qualitativo-quantitativo. Apresenta-se os dados socioeconômicos e demográficos do utentes. Resultados: Os achados nos informam que há prevalência de demanda via administrativa em relação a demanda via judicial. Os utentes têm dificuldade para efetuar o cadastro, renovação e o acesso ao medicamento; logo, apresentam dificuldades para adesão e continuidade ao tratamento. Conclusão: Infere-se que há dificuldades no acesso ao tratamento da FPI no Pará, sendo que a adesão ao tratamento e a continuidade estão prejudicadas pela inconstância na aquisição dos antifibróticos. Há prejuízos para a qualidade de vida dos utentes. Constatou-se altos gastos com a aquisição dos medicamentos antifibróticos.

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Section: Introductionunclassified

Itinerário de utentes com fibrose pulmonar idiopática atendidos via judicial ou administrativa pela Secretaria de Estado de Saúde do Pará

Kaminosono

Gillet

Sousa

et al. 2021

RSD

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Células NKT, macrófagos M2 e o desenvolvimento da fibrose pulmonar.

Grabarz¹

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Pulmonary fibrosis is a common pathway of various acute and chronic interstitial lung diseases that may result in abnormal wound healing of the lungs in response to damage of the alveolar epithelium. During this process, there is excessive accumulation of extracellular matrix proteins (ECM) produced mainly by fibroblasts and myofibroblasts, leading to disruption of the alveolar walls, loss of tissue elasticity and loss of ventilatory function and gas exchange in the lungs. NKT cells are a major source of Th2 cytokines and may be crucial in the activation and polarization of macrophages to the M2 phenotype, contributing to pulmonary fibrogenesis. From these facts, our project has built a hypothesis that NKT cells could influence the development of pulmonary fibrosis via modulation of macrophage activation. Thus, in an experimental model of pulmonary fibrosis induced by bleomycin, we studied the interaction between NKT cells and alveolar macrophages. For this, wild and knockout invariant NKT cells (Jα18-/-) mice were subjected to the protocol of bleomycin induced pulmonary fibrosis. NKT cells were activated with two different ligands: the α-galactosylceramide and sulfatide. Participation of different cell populations, especially NKT cells and macrophages, as well as the inflammatory infiltrate and deposition of collagen production and gene expression of cytokines involved in the inflammation and fibrosis, were evaluated by flow cytometry and qPCR. The results indicate that the Jα18-/mice, as well as the experimental groups receiving agonists for NKT cells, showed protection against lung fibrosis, since there was less hydroxyproline synthesis and deposition of collagen. In these groups, there is also the decrease of pro-fibrotic cytokines and maintaining of the M1 phenotype of macrophages in lung tissue. Our data suggest that NKT cells may be one of the responsible to modulate the pattern of the inflammatory response in this experimental model, contributing to the development of fibrosis.

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Current and emerging drugs for idiopathic pulmonary fibrosis

Gharaee−Kermani

Thannickal

et al. 2007

Expert Opinion on Emerging Drugs

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Idiopathic pulmonary fibrosis is a chronic, progressive and often fatal form of interstitial lung disease. It is characterized by injury with loss of lung epithelial cells and abnormal tissue repair, resulting in abnormal accumulation of fibroblasts and myofibroblasts, deposition of extracellular matrix and distortion of lung architecture, leading to respiratory failure. This lethal lung disorder continues to pose major clinical challenges as an effective therapeutic regimen has yet to be developed. In this report, therapeutic strategies are reviewed, including the use of antifibrotic agents, inhibition of cytokines, leukotrienes and cytokines receptors, and molecular targeting of specific signaling pathways during fibrotic processes and angiogenesis. This article examines the body of evidence supporting present therapies and reviews the newer agents being tested in patients with idiopathic pulmonary fibrosis.

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Fibrose pulmonar idiopática: uma década de progressos

Cited by 3 publications

References 54 publications

Itinerário de utentes com fibrose pulmonar idiopática atendidos via judicial ou administrativa pela Secretaria de Estado de Saúde do Pará

Itinerário de utentes com fibrose pulmonar idiopática atendidos via judicial ou administrativa pela Secretaria de Estado de Saúde do Pará

Células NKT, macrófagos M2 e o desenvolvimento da fibrose pulmonar.

Current and emerging drugs for idiopathic pulmonary fibrosis

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