High-Dose Intravenous Epoprostenol and Adjunct Therapies for Refractory Raynaud’s Phenomenon Associated with Anti-Jo-1 Antibodies: A Case Report
Andy Pan
Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada, Department of Emergency Medicine, The Ottawa Hospital, Ottawa, Ontario, Canada and Division of Critical Care Medicine, Hôpital Montfort, Ottawa, Ontario, Canada.
Imran Khan *
Department of Pharmacy, Hôpital Montfort, Ottawa, Ontario, Canada, School of Pharmaceutical Sciences, University of Ottawa, Ottawa, Ontario, Canada and Institut du Savoir Montfort, Hôpital Montfort, Ottawa, Ontario, Canada.
Marlene Mansour
Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada and Division of Critical Care Medicine, Hôpital Montfort, Ottawa, Ontario, Canada.
Bernadett Kovacs
Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada, Division of Critical Care Medicine, Hôpital Montfort, Ottawa, Ontario, Canada and Institut du Savoir Montfort, Hôpital Montfort, Ottawa, Ontario, Canada.
Caitlin Richler
Department of Pharmacy, Hôpital Montfort, Ottawa, Ontario, Canada and School of Pharmaceutical Sciences, University of Ottawa, Ottawa, Ontario, Canada.
Christine Landry
Department of Pharmacy, Hôpital Montfort, Ottawa, Ontario, Canada, School of Pharmaceutical Sciences, University of Ottawa, Ottawa, Ontario, Canada and Institut du Savoir Montfort, Hôpital Montfort, Ottawa, Ontario, Canada.
Kimberley Do
Department of Pharmacy, Hôpital Montfort, Ottawa, Ontario, Canada, School of Pharmaceutical Sciences, University of Ottawa, Ottawa, Ontario, Canada and Institut du Savoir Montfort, Hôpital Montfort, Ottawa, Ontario, Canada.
Sydney Morin
Department of Pharmacy, Hôpital Montfort, Ottawa, Ontario, Canada and School of Pharmaceutical Sciences, University of Ottawa, Ottawa, Ontario, Canada.
Deb Purkayastha
Department of Pharmacy, Hôpital Montfort, Ottawa, Ontario, Canada and School of Pharmaceutical Sciences, University of Ottawa, Ottawa, Ontario, Canada.
Melissa Varsava
Department of Pharmacy, Hôpital Montfort, Ottawa, Ontario, Canada and School of Pharmaceutical Sciences, University of Ottawa, Ottawa, Ontario, Canada.
Emma Baun
Programme de Pharmacie, Université de Lyon, Lyon, France.
Pierre Thabet
Department of Pharmacy, Hôpital Montfort, Ottawa, Ontario, Canada, School of Pharmaceutical Sciences, University of Ottawa, Ottawa, Ontario, Canada and Institut du Savoir Montfort, Hôpital Montfort, Ottawa, Ontario, Canada.
*Author to whom correspondence should be addressed.
Abstract
This case report highlights the use of intravenous high-dose Epoprostenol for managing severe digital ischemia in a patient diagnosed with antisynthetase syndrome (ASyS) exhibiting anti-Jo1 antibodies positivity, emphasizing its vasodilatory benefits and challenges with tolerability at higher doses. A multi-modal treatment strategy, including calcium channel blockers, phosphodiesterase inhibitors, hyperbaric oxygen therapy, and prostacyclin analogs, was critical for optimizing outcomes in refractory Raynaud’s phenomenon. This case underscores the importance of individualized treatment regimens and the need for ongoing monitoring and adjustments to balance therapeutic efficacy with patient safety. The report contributes to the limited literature on the management of severe digital ischemia in ASyS and emphasizes the potential role of adjunct therapies in improving patient outcomes.
Keywords: Antisynthetase syndrome, digital ischemia, adjunct therapies, hypotension