Guidelines or guidance for better idiopathic pulmonary fibrosis management?

Idiopathic pulmonary fibrosis (IPF) is a rare fibrotic interstitial lung disease with a relentlessly progressive course and fatal outcome. Guidelines summarizing the current evidence and providing evidence-based recommendations for the treatment of rare diseases such as IPF are important since individual physicians often have limited experience. Nevertheless, the available evidence is often scarce and, therefore, evidence-based recommendations are prone to being vague or with low confidence, thus creating uncertainty instead of guidance. Moreover, the effect of guidelines themselves on clinical practice has not been sufficiently evaluated. On the other hand, expert opinion may be biased and lead to the misinterpretation of evidence, resulting in misleading recommendations and a potential harm to patients. This editorial focuses on the advantages and disadvantages of evidence-based guidelines and professional experience in the context of a rare disease such as IPF and tries to assess the optimum combination of both approaches.