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Reversible Platypnea-Orthodeoxia Syndrome from Ventilation-Perfusion Mismatch in Interstitial Lung Disease: A Novel Etiology.

Platypnea-orthodeoxia syndrome is most commonly associated with intracardiac shunts (e.g., atrial septal defect) and intrapulmonary shunts (e.g., hepatopulmonary syndrome). We report a case of platypnea–orthodeoxia syndrome secondary to interstitial lung disease associated with the antisynthetase syndrome. The likely mechanism is predominant lower lung parenchymal disease resulting in significant ventilation–perfusion mismatch on sitting up from a supine position.