Because of the critical role of fibrillin-1 in sequestering latent TGF-β in the ECM and in stabilizing blood vessel wall [6], a normal deposition of fibrillin-1 by B-MVECs, as observed upon challenge with CYC-treated SSc sera, might contribute either indirectly to the control of fibroblast activation, hence limiting fibrosis, or directly to microvascular deremodeling. Here, FBN1 is linked to systemic sclerosis.