DETECTION AND TREATMENT OF IDIOPATHIC PULMONARY FIBROSIS

The present disclosure relates generally to compositions and methods for diagnosing and treating idiopathic pulmonary fibrosis (IPF). Also, methods for identifying IPF disease severity and likelihood for disease progression. An IPF patient can be identified when the CSF1 concentration or soluble CSF1R concentration in a blood sample is decreased as compared to a reference blood sample from a reference human subject not having IPF, and/or when the soluble CSF1R concentration in a bronchoalveolar lavage (BAL) fluid sample is increased as compared to a reference BAL fluid sample from the reference human subject. Once identified, the patient can be treated with, for instance, CSF1 or CSF1R inhibitors, such as antibodies. Moreover, the IPF patient soluble CSF1R level in blood or BAL samples can also be used to assess the severity of disease, and to both monitor disease progression and treatment outcomes.