Hypersensitivity Pneumonitis in Progressive Fibrosing Interstitial Lung Disease

This protocol addresses hypersensitivity pneumonitis (HP) in the specific setting of progressive fibrosing interstitial lung disease — including fibrotic HP with a progressive phenotype, a clinically distinct presentation that warrants a structured, evidence-based approach.

Clinical Scenario

The patient presents with hypersensitivity pneumonitis exhibiting a progressive fibrosing ILD course, or with fibrotic HP and a documented progressive phenotype. This subset of HP carries particular management considerations beyond the general HP population.

Management Approach (Partial Overview)

A foundational step in management centres on identifying the inciting antigen and implementing antigen avoidance wherever possible. This step is critical, though the complete protocol includes further structured guidance beyond antigen avoidance alone.

The full regimen and sequential options are available via the link below.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1183/16000617.0169-2021

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.
Antigen avoidance should be implemented wherever possible.
Identification and elimination of the inciting antigen are critical to improving outcomes in patients with HP but can be difficult to achieve in practice.

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