Bird fancier's lung
ICD-10 J67.2 · ICD-11 CA70.2

Nonfibrotic hypersensitivity pneumonitis with features of inflammation: what to do when steroid-sparing immunosuppression has not worked

Clinical scenario

Bird fancier's lung presenting as hypersensitivity pneumonitis with inflammatory features — ground glass opacities on HRCT, bronchoalveolar lavage lymphocytosis, or acute/sub-acute symptom onset — with no pulmonary fibrosis on imaging or histopathology.

Previous treatment — goals not reached

A steroid-sparing immunosuppressive agent (mycophenolate mofetil, azathioprine, methotrexate, or cyclophosphamide) was added to allow corticosteroid weaning. The required goals — improvement in clinical parameters or disease stabilisation with maintenance of lung function (FVC, DLCO) — were not achieved, indicating the need to escalate.

Next-line approach

The next step involves a third-line immunosuppressive strategy using a biologic agent. The complete regimen and monitoring plan are detailed in the full protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1111/resp.14847

Moderate-to-high dose systemic corticosteroids would usually be considered for HP patients with features of inflammation (e.g., GGO on HRCT, BAL lymphocytosis, well-documented exposure with acute or sub-acute symptom onset).

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.

There is case series-level evidence only for third-line agents including rituximab in HP.

View source ↗