The use of steroids and mycophenolate mofetil in idiopathic and heritable pulmonary veno-occlusive disease: A case series: Is there a role for immunosuppression?

Pulmonary veno-occlusive disease (PVOD) is classified into idiopathic, heritable and associated with other conditions, most often connective tissue disease (CTD) and sarcoidosis. In patients with PVOD with a CTD, immunosuppressive therapies can improve symptoms, gas transfer, imaging and haemodynamics [1, 2]. Despite evidence for inflammation in PVOD pathophysiology [3, 4], including capillary immune complex and complement deposition [5], the role for immunosuppression in idiopathic or heritable forms of PVOD is uncertain. We present three patients with heritable and idiopathic PVOD who received immunosuppression for suspected immune dysregulation. Footnotes This manuscript has recently been accepted for publication in the European Respiratory Journal . It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article. Conflict of interest: Dr. Lindner has nothing to disclose. Conflict of interest: Dr. Bergbaum has nothing to disclose. Conflict of interest: Dr. Samaranayake has nothing to disclose. Conflict of interest: Dr. Pitcher reports personal fees from Janssen, during the conduct of the study. Conflict of interest: Dr. Weingart has nothing to disclose. Conflict of interest: Dr. Semple has nothing to disclose. Conflict of interest: Dr. Kokosi has nothing to disclose. Conflict of interest: Dr. Wells has nothing to disclose. Conflict of interest: Dr. Montani reports grants and personal fees from Janssen, grants and personal fees from Bayer, personal fees from GSK, personal fees from Pfizer, grants, personal fees and non-financial support from MSD, personal fees from Chiesi, personal fees from Boehrigher, non-financial support from Accerleron, outside the submitted work. Conflict of interest: Dr. Dimopoulos reports grants, personal fees and other from Janssen, during the conduct of the study. Conflict of interest: Dr. McCabe reports personal fees and other from Janssen, during the conduct of the study. Conflict of interest: Dr. Kempny has nothing to disclose. Conflict of interest: Dr. Harries has nothing to disclose. Conflict of interest: Dr. Orchard has nothing to disclose. Conflict of interest: Dr. Wort reports grants, personal fees from Janssen, grants and personal fees from MSD, grants from GSK, reseach grants from Janssen and Bayer, during the conduct of the study. Conflict of interest: Dr. Price reports personal fees from Janssen, during the conduct of the study.