Fibrotic hypersensitivity pneumonitis can follow a progressive fibrosing course, placing it within the spectrum of progressive fibrosing interstitial lung disease (PF-ILD). This protocol targets that specific phenotype — fibrotic HP with ongoing progression.
This protocol applies to patients with fibrotic hypersensitivity pneumonitis who develop a progressive phenotype — characterised by worsening fibrosis consistent with progressive fibrosing ILD. Anti-fibrotic therapy should be considered in this population.
For carefully selected patients in whom progressive fibrotic disease continues to advance, a definitive surgical option may be appropriate. The complete eligibility criteria, referral pathway, and the full structured regimen are available via the link below.
Anti-fibrotic therapy should be considered in patients with progressive fibrosing ILD.
Nintedanib, a tyrosine kinase inhibitor, is an approved treatment option for fibrotic HP with a progressive phenotype.
Lung transplant improves survival in select patients with progressive fibrotic ILDs.
DOI: 10.1183/16000617.0169-2021
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