Interstitial pneumonia with autoimmune features: from research classification to diagnosis

Area of Science:

• Pulmonology
• Rheumatology
• Immunology

Background:

• Interstitial pneumonia with autoimmune features (IPAF) was proposed in 2015 as a research classification.
• It aims to standardize nomenclature for patients with idiopathic interstitial pneumonia and connective tissue disease features.
• Clinical application and implications of IPAF remain uncertain.

Purpose of the Study:

• To provide a comprehensive overview of existing IPAF cohort studies.
• To discuss IPAF as a distinct diagnostic entity.
• To outline a suggested approach for patient management.

Main Methods:

• Review of published studies on IPAF cohorts.
• Analysis of heterogeneity within IPAF cohorts.
• Evaluation of preliminary data for patient management strategies.

Main Results:

• Significant heterogeneity exists among IPAF cohorts, resembling either CTD-ILD or idiopathic interstitial pneumonias (including IPF).
• Limited data is available to guide the management of IPAF patients.
• Preliminary findings support pragmatic management using existing diagnoses (IP or CTD-ILD).

Conclusions:

• The IPAF classification has aided research in this patient group.
• Unanswered questions persist regarding IPAF natural history and treatment response.
• Further research is required to validate management approaches for IPAF.