Reversible platypnea‐orthodeoxia syndrome induced by rapidly progressive interstitial pneumonia in a patient with polymyositis

Abstract

We report a case of platypnea-orthodeoxia that developed in a 64-year-old Japanese woman during an episode of rapidly progressive interstitial pneumonia with polymyositis. Pulmonary infiltrates were predominant in the bilateral lower lobes. The patient was treated successfully with early administration of immunosuppressive therapies and polymyxin B-immobilized fiber column-direct hemoperfusion, and her platypnea-orthodeoxia improved with resolution of the underlying parenchymal lung disease. Reports of platypnea-orthodeoxia syndrome with interstitial pneumonia are extremely rare. The recognition that platypnea-orthodeoxia syndrome may occur in multiple disease states, including interstitial pneumonia, is crucial to the understanding of this perplexing disorder.

Platypnea-orthodeoxia syndrome in idiopathic pulmonary fibrosis with Pneumocystis jiroveci pneumonia

“…Patients in whom this phenomenon has been documented in case reports had advanced emphysema, postpneumonectomy state,[ 3 ] amiodarone toxicity,[ 11 ] acute respiratory distress syndrome,[ 12 ] and recurrent pulmonary embolism. [ 13 ] Two cases of POS have been reported in patients with interstitial lung disease[ 4 5 ] and in two cases of pneumonia caused by opportunistic organisms such as P. jiroveci and cytomegalovirus. [ 6 7 ] A review of the literature revealed that POS has never been reported in a case of IPF or IPF complicated by PJP.…”

“…It has been attributed to an exaggerated ventilation-perfusion mismatch on assuming an upright posture due to gravity-dependent shift in perfusion to the lung bases. [ 3 5 6 12 ] The shunt in pulmonary conditions is thus intrapulmonary made worse with positional change to upright position.…”

“…[ 1 2 ] Most case reports have been in association with an intra-atrial communication such as an atrial septal defect (ASD) or a patent foramen ovale (PFO). [ 2 ] Observation of this unusual physiological abnormality in pulmonary conditions has been extremely rare, such reports being in association with emphysema, arteriovenous malformation, amiodarone toxicity,[ 3 ] rarely in interstitial lung disease,[ 4 5 ] and none in idiopathic pulmonary fibrosis (IPF). There are also two reports of POS occurring during pneumonia due to Pneumocystis jiroveci .…”

An extreme case of platypnoea-orthodeoxia syndrome

“…As in this case, POS is most commonly described in patients with right-to-left interatrial shunting. 1 Other recognised causes include pulmonary arteriovenous malformation, 2 large ventilation:perfusion mismatch of any cause, 3 and hepatopulmonary syndrome. 4 When one considers the total number of reported cases of POS (188) 2 compared with the prevalence of PFO (approximately 10%) in the adult population, it is apparent that the presence of an interatrial defect is not in itself sufficient to bring about POS.…”