Hypersensitivity pneumonitis
ICD-10 J67 · ICD-11 CA70

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

This protocol addresses management of hypersensitivity pneumonitis (HP) when the disease has taken a fibrotic course and is accompanied by severe lung function impairment or evidence of active progression — a subset in which standard antigen avoidance alone may be insufficient.

Clinical scenario: Fibrotic hypersensitivity pneumonitis with severe lung function impairment or progressive disease. In this presentation, the clinical picture is more challenging and may warrant a structured pharmacological approach beyond environmental measures.

Treatment approach

In this setting, immunosuppressive therapy is among the strategies that may be considered. The structured regimen — covering which agents apply, how to sequence them, and the monitoring framework — is available in the full protocol.

Treatment goal: improvement in lung function parameters (DLCO or FVC) after a sustained course of therapy.

Instant Access to Structured Evidence-Based Regimens

References

In cases of fibrotic HP and severe or progressive disease, immunosuppressive therapy may be considered.

Some retrospective analyses have shown an improvement in DLCO or FVC after a year of treatment with mycophenolate mofetil (MMF) or azathioprine.

DOI: 10.1183/16000617.0169-2021

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