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    These findings are significant, as there are currently no approved disease-specific therapies for patients with PH complicating IPF. Keywords: pulmonary hypertension, pulmonary fibrosis, myeloid-derived suppressor cell, C-X-C motif chemokine receptor 2, IL-8 Clinical Relevance This study demonstrates for the first time that, in models of pulmonary hypertension, myeloid-derived suppressor cell trafficking to the lung, mediated by C-X-C motif chemokine receptor 2, is increased.

    Furthermore, a C-X-C motif chemokine receptor 2 antagonist inhibits granulocytic myeloid-derived suppressor cell accumulation within the lung, preventing development of PH.

    Pulmonary hypertension PH associated with IPF is the most frequent comorbidity contributing to intensive use of hospital and emergency room services by this patient population 2.

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    Furthermore, IPF complicated by PH is associated with significant impairment in quality-of-life, and a fourfold increase risk of death 4, compared with those patients with IPF alone. Despite the poor prognosis, there are currently no approved treatments for secondary PH.

    Thus, there is a substantial need for novel and clinically translatable pharmacologic targets in the field of pulmonary vascular biology.


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