Interstitial Pneumonia with Autoimmune Features (IPAF): A Single-Centre, Prospective Study.

Area of Science:

• Pulmonology
• Rheumatology
• Immunology

Background:

• Interstitial pneumonia with autoimmune features (IPAF) describes interstitial lung disease in patients lacking criteria for specific connective tissue diseases.
• Understanding IPAF characteristics, progression, and treatment response is crucial for patient management.

Purpose of the Study:

• To investigate the clinical characteristics, disease progression, treatment response, and complications in patients with IPAF over a one-year follow-up period.

Main Methods:

• A cohort of 39 IPAF patients was studied, documenting clinical and laboratory findings, comorbidities, medications, pulmonary function tests (PFTs), and chest HRCT.
• Data collection included a one-year follow-up to assess disease progression and complications.

Main Results:

• The average age of IPAF diagnosis was 63.2 years, with 62% being female. Arthritis (82%) and rash (54%) were common features.
• Antinuclear antibodies (ANA) and non-specific interstitial pneumonia (NSIP) patterns were most prevalent.
• Pulmonary function tests stabilized or improved in 79.5% of patients at 12 months. Infections occurred in 23.1% during the first semester, primarily in patients with non-usual interstitial pneumonia (non-UIP) patterns.

Conclusions:

• Arthritis and rash are frequent in IPAF, suggesting potential inclusion in diagnostic criteria.
• A significant majority (nearly 80%) of IPAF patients experienced stable or improved lung function after one year.
• Infections were more common in the first treatment semester and in non-UIP radiological patterns, possibly linked to higher corticosteroid doses.