Late Breaking Abstract - Autoantibody reduction treatment for acute exacerbations of idiopathic pulmonary fibrosis.

Résumé fourni par la source

Background: No medical treatment has proven benefit for acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF) and this syndrome has a very high mortality. Two prior observational studies suggested autoantibody reduction therapy (AART) has efficacy for AE-IPF, but this approach had not yet been tested by randomized clinical trial (RCT). Methods: AE-IPF patients with no evidence of autoimmune diseases were stratified by their use of supplemental oxygen and antifibrotic medication at presentation and then randomized (2:1) to either AART (total plasma exchange plus rituximab plus intravenous immunoglobulin) or Treatment as Usual (TAU) (corticosteroids and antibiotics). The primary endpoint was 180-day survival. Secondary endpoints included treatment effects on six-minute walk distances (6MWD). Results: Target enrollment (n=51) was met with 33 AART participants and 18 TAU. Actuarial 180-day survival was 44+9% after AART compared to 7+6% among TAU (p=0.036). Among patients requiring O2 at or below the stratification threshold (<5L/min by simple nasal cannula), AART survival (n=10) was 80+13% compared to 19+17% for TAU (p=0.016). Conversely, only one of the 25 subjects who required high-flow nasal cannula O2 at randomization (an AART) survived 180 days. 6MWD increased after AART by 73+160 feet (p=0.014) and was significantly greater (318+406 feet) than among TAU (p=0.021) in whom walk distances had decreased to 54+144 feet. Conclusions: AART mproved survival and exercise tolerance of AE-IPF patients with mild-moderate hypoxemia. This is the first medical therapy shown by RCT to benefit AE-IPF patients. These results also implicate humoral autoimmunity in AE-IPF pathogenesis.

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