Hypersensitivity Pneumonitis
ICD-10 J67 · ICD-11 CA70

Treatment of Hypersensitivity Pneumonitis in Fibrotic Disease with Severe or Progressive Lung Impairment

Managing fibrotic hypersensitivity pneumonitis (fibrotic HP) becomes significantly more complex when patients present with severe lung function impairment or a progressive disease course. This subset requires a structured, evidence-based approach that addresses both the underlying driver and the severity of functional decline.

Clinical scenario

This protocol is for patients with fibrotic hypersensitivity pneumonitis who have severe lung function impairment or ongoing disease progression — a high-acuity presentation where standard considerations alone are insufficient and broader management strategies come into play.

Approach

A foundational priority in this setting is the identification and elimination of the inciting antigen, with antigen avoidance implemented wherever feasible — though this can be difficult to achieve in practice. The complete protocol defines the structured steps that follow this essential first measure, including what is considered when avoidance alone is insufficient.

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References

DOI: 10.1183/16000617.0169-2021
  • In cases of fibrotic HP and severe or progressive disease, immunosuppressive therapy may be considered.
  • 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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