The PROFIBRO Survey – Progressive Pulmonary Fibrosis Management in Real-World Setting

Progressive Pulmonary Fibrosis Management in Real-World Setting – The PROFIBRO Survey

Authors & Institutions

M. Mehta¹, Sujeet R², Y. Sivagnaname³, V.K. Chennamchetty⁴, S. Singh⁵, M. Shah⁶, S. Krishnan⁷, K. Krishnamurthy⁸, D. Muthreja⁹, R. Jalan¹⁰, S. Tale¹¹, M. Lopez¹, S. Mohanasundaram¹, J. Gogtay¹
¹Cipla Ltd, India; other co-authors from major hospitals across India.

Introduction

• Progressive pulmonary fibrosis (PPF) constitutes 13–40% of fibrotic interstitial lung diseases (F-ILD).
• Limited Indian data available on real-world PPF management.

Objective

• To understand management patterns of PPF in real-world clinical settings.

Methodology

• Clinicians attending CME programs (Jan–Dec 2024) on respiratory diseases completed a survey questionnaire.
• 117 clinicians from 16 Indian states participated (87% pulmonologists).
• Results expressed as percentages based on responses.

Results

PPF Diagnosis

• 50% based diagnosis on clinical judgment with radiologic and lung function findings.
• 42%, 6%, and 2% followed 2022 ATS/ERS/ALAT/JRS, INBUILD, and RELIEF criteria respectively.

Prevalence of PPF among F-ILD patients

• 48% clinicians: 10–30% of their F-ILD patients develop PPF.
• 32%: <10% (Figure 1).

Diagnosis and Monitoring

• 39% labelled F-ILD as PPF regardless of fibrosis duration.
  • 27% labelled after 12 months, 21% after 6 months.
• Commonly associated ILDs:
  • F-HP (85%)
  • RA-ILD (72%)
• Preferred monitoring tools:
  • FVC (89%), HRCT (88%), 6MWD (84%)

Treatment Practices

Before Progression

• 63% used prednisolone in >50% of non-IPF ILD patients before progression.
• 44% preferred combination of mycophenolate + low-dose prednisolone (Figure 2).

After Progression

• Most frequent additional treatments (>50% of cases) (Figure 3):
  • Nintedanib (70%)
  • Increase dose of prednisolone (49%)
  • Pirfenidone (39%)
  • Mycophenolate ± prednisolone (27%)

Antifibrotic Use

• 44% clinicians considered antifibrotic combination therapy when progression occurred despite single antifibrotic.
• 13% used combination therapy upfront.
• 12% when single antifibrotic not tolerated (Figure 4).

Pulmonary Rehabilitation & Palliative Care

• 61% offered Pulmonary Rehabilitation (PR) routinely.
• 71% continued PR as long as the patient wished.
• Palliative care initiated in:
  • 15% (post-treatment initiation)
  • 22% (symptoms worsen despite treatment)
  • 27% (NIV-dependent), 26% (O₂-dependent)

Lung Transplant Referral

• Done early if no contraindications (29%).
• 27%, 11%, and 13% referred only in specific scenarios (refractory hypoxemia/NIV dependence).
• 20% did not refer for transplant.

Summary & Conclusions

• Steroid dose escalation is a common first step before antifibrotic initiation.
• Nintedanib – preferred antifibrotic in established PPF.
• Nearly half the clinicians used dual antifibrotic therapy for progression on single antifibrotic.
• PPF management in India shows reliance on clinical judgment, steroids, and gradual antifibrotic introduction.

Thanks