title: Immunosuppressant-induced endothelial damage and pulmonary arterial hypertension.
abstract: Cyclosporine A, used to prevent graft-versus-host-disease, is known to induce endothelial injury. Endothelial dysfunction is an important feature of pulmonary arterial hypertension (PAH). In this article, we describe 2 children who developed cyclosporine-induced acute respiratory distress syndrome. Lung biopsy showed patchy loss of endothelial caveolin-1 and von Willebrand factor to occur early. Significant loss of endothelial caveolin-1 was associated with robust expression of caveolin-1 in smooth muscle cells with subsequent neointima formation leading to fatal PAH. Thus, patients who develop acute respiratory distress syndrome after immunosuppressive therapy are at risk of developing PAH.
authors.affiliation: Section of Cardiology, Department of Pediatrics, Maria Fareri Children's Hospital at Westchester Medical Center, New York Medical College, Valhalla, NY, USA. rajamma_mathew@NYMC.edu
authors.forename: Rajamma
authors.initials: R
authors.lastname: Mathew
authors.forename: Jing
authors.initials: J
authors.lastname: Huang
authors.forename: Umadevi S
authors.initials: US
authors.lastname: Katta
authors.forename: Usha
authors.initials: U
authors.lastname: Krishnan
authors.forename: Claudio
authors.initials: C
authors.lastname: Sandoval
authors.forename: Michael H
authors.initials: MH
authors.lastname: Gewitz
mesh_terms: D002675:Child, Preschool; D016572:Cyclosporine / Q000009:adverse effects / Q000494:pharmacology*; D004730:Endothelium, Vascular / Q000187:drug effects* / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000503:physiopathology; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000494:pharmacology*; D007223:Infant; D008297:Male; D012128:Respiratory Distress Syndrome / Q000188:drug therapy* / Q000503:physiopathology; D012307:Risk Factors
chemical_list: D007166:Immunosuppressive Agents; D016572:Cyclosporine