Historia y metodología de las primeras guías chilenas de fibrosis pulmonar idiopática 2019 / History and methodology of the first Chilean idiopathic pulmonary fibrosis guidelines, 2019

Desde 2017 los miembros de la Comisión de Enfermedades Pulmonares Intersticiales Difusas de la Sociedad Chilena de Enfermedades Respiratorias hemos trabajado en la elaboración de las primeras guías de fibrosis pulmonar idiopática (FPI) del país, necesidad evidente para fomentar el diagnóstico precoz y adecuado de la enfermedad y establecer una base para posible incorporación de su cuidado en cobertura de seguros de salud especiales. Se elaboraron 5 preguntas de revisión de evidencia y el resto se trabajó en formato de preguntas de contexto. Un grupo de metodólogos graduaron la evidencia siguiendo la metodología GRADE.

Since 2017, the members of the Commission of Diffuse Interstitial Lung Diseases of the Chilean Society of Respiratory Diseases have worked in the development of the first guidelines of idiopathic pulmonary fibrosis (IPF) in the country, an obvious need to encourage early and adequate diagnosis of the disease and establish a basis for possible incorporation of IPF patients care into special health insurance coverage. Five evidence review questions were prepared and the remainder were worked out in context question format. A group of methodologists graduated the evidence following the GRADE methodology.

What's in a name? That which we call IPF, by any other name would act the same.

Idiopathic pulmonary fibrosis (IPF) remains a truly idiopathic fibrotic disease, with a modest genetic predilection and candidate triggers but no overall explanation for the development of disease in non-familial cases. Agreement on terminology has contributed to major clinical and translational advances since the millennium. It is likely that the entity currently captured by the term "IPF" will be radically reclassified over the next decade, either through "splitting" (into IPF subgroups responding selectively to individual disease-modifying agents) or through "lumping" of IPF with other forms of progressive fibrotic lung disease (with shared pathogenetic mechanisms and IPF-like disease behaviour). In this perspective, we summarise the clinical and pathogenetic justification for a focus on "the progressive fibrotic phenotype" in future clinical and translational research. By this means, we can hope to address the needs of non-IPF patients with inexorably progressive fibrotic disease, currently disenfranchised by lack of access to agents that are efficacious in IPF. In this regard, ongoing trials of anti-fibrotic therapies in non-IPF patients with progressive fibrosis may be highly influential. Future revision of IPF nomenclature may be warranted if there are major conceptual changes but without compelling justification, the benefits of renaming IPF are likely to be outweighed by the resulting confusion.

Pharmacological treatment of idiopathic pulmonary fibrosis: from the past to the future.

During the past decade important progress has been made regarding the pathogenesis of idiopathic pulmonary fibrosis (IPF), which is the most devastating form of idiopathic interstitial pneumonia with a median survival of 3 years. The knowledge gained has been used to design multicentre, randomised, placebo-controlled trials in order to investigate agents with different mechanisms of action. Encouraging results have led to licensing of the first IPF-specific drug, pirfenidone. However, the road to successful treatment is still long. The main aim for the future should be the careful design of clinical trials, by choosing the most clinically meaningful end-point and keeping in mind that combination of various agents may be more effective. This approach has been used in the treatment of lung cancer with which IPF presents many similarities.