In 2005, the antibody was tested in IPF patients in a phase I trial (NCT00125385); however, the result remains unpublished. The phase I trial (NCT01291784) of fresolimumab for myelofibrosis was terminated owing to its cardiotoxicity. Furthermore, the antibody also failed to meet the primary or secondary endpoints in treatment of SR-FSGS. Another small open-label trial (NCT01284322), fresolimumab significantly decreased the expression of THBS1 and COMP, and improved mRSS in SSc patients. However, no follow-up study has been reported. Here, COMP is linked to myelofibrosis.