التهاب الرئة التحسسي الليفي: ماذا تفعل عندما يفشل العلاج المثبط للمناعة في استقرار وظائف الرئة

References

DOI: 10.1111/resp.14847

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 fibrotic HP who progress despite antigen remediation, with or without immunosuppression, are considered to have PPF disease behaviour.

In such cases, there is good evidence for the introduction of antifibrotic therapy, as supported by the ATS/ERS/JRS/ALAT clinical guidelines for PPF.

Both nintedanib and pirfenidone have had regulatory body approval and subsidization for the indication of IPF in Australia and New Zealand for several years.

The INBUILD trial recruited patients with progressive fibrosing ILDs other than IPF (including fibrotic HP), demonstrating reduction in the rate of FVC decline in the nintedanib arm compared to placebo at 52 weeks, with a similar efficacy to IPF.