Interstitial pneumonia with autoimmune features

Area of Science:

• Pulmonology
• Rheumatology
• Immunology

Background:

• Some patients with idiopathic interstitial pneumonias (IIPs) exhibit autoimmune features but do not meet criteria for connective tissue disease (CTD).
• Previous classifications like undifferentiated connective tissue disease (UCTD) and autoimmune-featured interstitial lung disease (AIF-ILD) lacked uniformity.
• The European Respiratory Society/American Thoracic Society proposed 'interstitial pneumonia with autoimmune features' (IPAF) to standardize classification.

Purpose of the Study:

• To establish consensus nomenclature and classification criteria for patients with IIP and autoimmune features.
• To define the clinical entity of IPAF.

Main Methods:

• Review and synthesis of existing literature and classification criteria.
• Proposal of a consensus definition and criteria by a joint task force.

Main Results:

• IPAF represents a significant proportion of interstitial lung disease (ILD) cases, ranging from 7.3% to 34.1%.
• Key aspects like survival, progression to CTD, and optimal treatment remain to be elucidated.
• The distinct categorization of IPAF within ILD requires further investigation.

Conclusions:

• IPAF provides a standardized term for IIP patients with autoimmune features.
• Prospective studies are essential to understand IPAF's clinical course, treatment, and its relationship to established CTDs.
• Multidisciplinary collaboration, including rheumatologists, is crucial for advancing IPAF research and patient care.