![]() ![]() |
Related Articles |
In principle, accurate guideline recommendations should lead to optimal management based on a secure diagnosis. However, current IPF diagnostic guidelines do not meet the needs of a major sub-group (possibly the majority) of patients with idiopathic pulmonary fibrosis (IPF). A great many IPF patients have HRCT appearances of "possible UIP".
A surgical biopsy is very often impracticable due to age, disease severity, co-morbidities or patient refusal. A guideline-based diagnosis cannot be made in these patients, although the diagnosis is often obvious. Inflexible diagnostic criteria, although essential for treatment trials, must necessarily be structured around an inflexible diagnostic algorithm. With this approach, non-standardised information (i.e. not available in all patients) must be omitted, including observed disease behaviour prior to and on treatment, findings on bronchoalveolar lavage, likelihoods in relation to age and a wealth of ancillary clinical information.
However, when a diagnosis cannot be made using guideline criteria, a probable or highly probable "working diagnosis" of IPF can and should be made in most IPF patients by means of clinical reasoning, integrating all available non-standardised information.
![]() ![]() |
Related Articles |
Evidence-based guidelines have undergone an incredible transformation over the last number of years. Significant advances include explicit linkages of systematic evidence summaries to the strength and direction of recommendations, consideration of all patient-important factors, transparent reporting of the recommendation generation process including conflict of interest management strategies and the production of clinical practice guidelines which use simple and clear language.
The Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology provides a framework for guideline development and was employed to produce the recently published ATS/ERS/JRS/ALAT update on treatment for idiopathic pulmonary fibrosis (IPF). Herein we discuss the advantages of using an evidence-based approach for guideline development using the IPF process and resultant document as an example.
![]() ![]() |
Related Articles |
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.Please see related commentary articles: http://dx.doi.org/10.1186/s12916-016-0562-1 and http://dx.doi.org/10.1186/s12916-016-0563-0.