Autoantibody Reduction for Acute Exacerbations of Idiopathic Pulmonary Fibrosis
Status:
Recruiting
Trial end date:
2022-09-30
Target enrollment:
Participant gender:
Summary
Acute exacerbations (AE) are a dreaded manifestation of idiopathic pulmonary fibrosis (IPF)
that presents with rapidly worsening respiratory function over days to weeks. AE account for
about 1/2 the deaths in IPF patients, and are refractory to all medical therapies attempted
to date.
Considerable preliminary data shows pathological B-cell abnormalities and autoantibodies are
present in AE-IPF and associated with disease severity.
The experimental therapy here (therapeutic plasma exchange plus rituximab plus intravenous
immunoglobulin) is mechanistically targeted to ameliorate autoantibody-mediated pulmonary
injury. Anecdotal pilot studies indicate these treatments have significant benefit for a
disease syndrome that has, until now, been almost invariably inexorable. This clinical trial
has the potential to profoundly affect current paradigms and treatment approaches to patients
with AE-IPF.
Phase:
Phase 2
Details
Lead Sponsor:
University of Alabama at Birmingham
Collaborators:
Brigham and Women's Hospital National Heart, Lung, and Blood Institute (NHLBI) Temple University University of Pittsburgh