Bird Fancier's Lung with Hypersensitivity Pneumonitis and Pulmonary Fibrosis on HRCT: What to Do When Antifibrotic Therapy Has Not Achieved Disease Stabilisation

Clinical scenario

This protocol applies to Bird fancier's lung presenting as hypersensitivity pneumonitis (HP) with established pulmonary fibrosis on HRCT — evidenced by reticulation, traction bronchiectasis, and honeycombing. These imaging findings define a fibrotic (PPF) phenotype that carries distinct prognostic and treatment implications compared to non-fibrotic HP.

Previous treatment — failure condition

Antifibrotic therapy with nintedanib or pirfenidone was used to manage the progressive fibrotic disease behaviour. The intended goal was to reduce the rate of decline in forced vital capacity and achieve disease stabilisation. This protocol addresses the situation where that goal has not been reached and the disease continues to progress despite antifibrotic treatment.

Next-step approach (partial)

When fibrotic HP continues to progress and mortality risk becomes high, the management pathway shifts to a surgical approach. The specific eligibility criteria, referral timing, and full decision algorithm remain in the structured protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1111/resp.14847

Patients with HP may also display features of fibrosis, including reticulation, traction bronchiectasis and honeycombing (Figure 2B).

Recently published international diagnostic guidelines recommend HP classification into fibrotic or nonfibrotic phenotypes based on the presence or absence of radiological and/or histopathological fibrosis, supported by the observation that pulmonary fibrosis conveys important prognostic and treatment implications.

Patients with HP that have a PPF phenotype may have a trajectory similar to IPF and should be referred early for transplant consideration.

Lung transplant should be considered for patients with >50% risk of dying from their disease within 2 years, and >80% likelihood of 5-year post-transplant survival provided there is adequate graft function if transplanted.

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