"Any fool can make a rule and any fool will mind it"

Area of Science:

• Pulmonology
• Radiology
• Pathology

Background:

• Idiopathic pulmonary fibrosis (IPF) diagnosis relies on specific guidelines.
• Current guidelines inadequately address patients with high-resolution computed tomography (HRCT) findings of "possible usual interstitial pneumonia (UIP)".

Purpose of the Study:

• To evaluate the limitations of current IPF diagnostic guidelines.
• To propose an alternative approach for diagnosing IPF in challenging cases.

Main Methods:

• Analysis of diagnostic challenges in IPF patients with "possible UIP" on HRCT.
• Review of limitations imposed by inflexible diagnostic algorithms in guideline-based diagnosis.
• Exploration of clinical reasoning integrating non-standardized data.

Main Results:

• A significant subgroup of IPF patients, particularly those with "possible UIP" on HRCT, cannot achieve a guideline-based diagnosis.
• Surgical lung biopsy is often not feasible due to patient factors (age, severity, comorbidities, refusal).
• Inflexible criteria exclude valuable non-standardized information like disease behavior and ancillary clinical data.

Conclusions:

• Current IPF diagnostic guidelines are insufficient for a substantial patient population.
• Clinical reasoning, integrating all available information, is essential for establishing a probable IPF diagnosis when guideline criteria are not met.
• A probable or highly probable working diagnosis of IPF should be made using comprehensive clinical judgment.