Methods for the diagnosis and treatment of pulmonary fibrosis in subjects with hermansky pudlak syndrome

摘要

Described herein are methods, assays, and systems related to the prognosis, diagnosis, and treatment of Hermansky Pudlak Syndrome (HPS) and, e.g., pulmonary fibrosis in a subject. As described herein, subjects with HPS who have, will develop, or are most at risk or developing pulmonary fibrosis have elevated levels of CHI3L1. In some embodiments, the methods comprise administering an agonist of IL-13Rα2 or an inhibitor of CRTH2 to the subject.