Identification of UIP pattern fibrosis progression after antifibrotic therapy

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Introduction & Background Forced vital capacity (FVC), gas transfer (DLCO), echocardiographic findings, radiological patterns (UIP, probable UIP, NSIP), and prognostic scores (GAP) provide key insights into interstitial lung disease (ILD) outcomes. Aims & Objectives This study assesses whether these factors predict prognosis following antifibrotic therapy initiation after multidisciplinary team (MDT) discussions. Methods: We retrospectively analysed patients eligible for antifibrotic therapy post-MDT discussion (Jan 2020–Dec 2022) with follow-up until Aug 2024. Demographics and clinical data were collected. Annualized FVC and DLCO changes were calculated, and pulmonary artery pressure (PAP) was estimated via echocardiography. Results: 134 consecutive patients (mean age: 71 years; 85 ♂) were included. Baseline pulmonary function tests showed a mean predicted FVC of 84% ± 20 and DLCO of 44% ± 15. GAP stages: 1 (n=38), 2 (n=42), 3 (n=7). Annualized FVC decline: >10% in 32, 5–10% in 18, and <5% in 43. DLCO declined >10% in 24 and <10% in 34. PAP was <30 mmHg in 30 and >30 mmHg in 34. At census, 66 were alive, 67 had died. One-year survival: 80%; three-year: 48%. Patients with PAP >30 mmHg had worse three-year survival (26%) vs. PAP <30 mmHg (60%, p<0.05). No significant survival associations were found with GAP score, FVC, FVC change, DLCO, DLCO change, radiological pattern, age, sex, or smoking. Conclusion: Post-antifibrotic therapy mortality varied significantly. Pulmonary hypertension correlated with poorer survival, while other physiological markers lacked prognostic value. The holistic MDT approach may influence outcomes, warranting further research to refine treatment strategies.

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