Fibrotic Hypersensitivity Pneumonitis with Severe or Progressive Disease — What to Do After Immunosuppressive Therapy Has Failed
This protocol applies to patients with fibrotic hypersensitivity pneumonitis (HP) who have severe lung function impairment or progressive disease and who have not responded adequately to first-line immunosuppressive treatment.
Clinical Scenario
Fibrotic HP in which disease severity is significant or the course is progressive — a setting where standard immunosuppressive measures alone have proven insufficient to stabilise or reverse functional decline.
Previous Treatment — Failure Condition
Patients who received immunosuppressive therapy with mycophenolate mofetil (MMF) or azathioprine and did not achieve improvement in DLCO or FVC after one year of treatment meet the failure threshold that escalates care to this next-line protocol.
Next-Line Approach — Partial Overview
This protocol introduces a targeted biologic therapy for eligible patients in this refractory setting. The clinical goal is stabilization or improvement of FVC and DLCO. The complete agent selection, regimen structure, and monitoring guidance are available in the full protocol below.
References
DOI: 10.1183/16000617.0169-2021
In cases of fibrotic HP and severe or progressive disease, immunosuppressive therapy may be considered.
A retrospective study of 20 patients showed that treatment with rituximab for 6 months led to stabilization or improvement of FVC and DLCO in patients with HP whose disease had not improved following antigen avoidance and corticosteroid therapy.
View source ↗