Tofacitinib as a Steroid-Sparing Therapy in Pulmonary Sarcoidosis, an Open-Label Prospective Proof-of-Concept Study.

This is a prospective, open-label, proof-of-concept study of tofacitinib, a Janus kinase inhibitor, as a steroid-sparing therapy in corticosteroid-dependent pulmonary sarcoidosis. Five patients with corticosteroid-dependent pulmonary sarcoidosis were treated with tofacitinib 5 mg twice daily. The primary endpoint was a ≥ 50% reduction in corticosteroids at week 16 with no worsening in pulmonary function or respiratory symptoms. 60% of patients (3/5) met the primary endpoint. One patient was lost to follow up prior to steroid taper, and another was withdrawn due to worsening of known neurosarcoidosis. The three patients who met the primary endpoint each tapered to ≤ 5 mg/day prednisone, respiratory symptoms improved, and spirometry remained stable. In this proof-of-concept study, the addition of a JAK-inhibitor allowed 60% of patients with pulmonary sarcoidosis to successfully taper corticosteroids. JAK-inhibitors are a promising therapy for pulmonary sarcoidosis, which require further investigation in randomized trials.Trial Registration clinicaltrials.gov NCT03793439; registered Jan 4, 2019.

Refractory Hypoxemia in a Patient with Submassive Pulmonary Embolism and an Intracardiac Shunt: A Case Report and Review of the Literature.

INTRODUCTION: Acute pulmonary embolism is the third leading cause of cardiovascular death. Management options include anticoagulation with or without thrombolysis. Concurrent persistent hypoxemia should be a clue to the existence of an intracardiac shunt. CASE PRESENTATION: A 46-year-old man experienced acute hypoxemic respiratory failure requiring mechanical ventilation after anesthesia induction for elective hip arthroplasty. He was found to have submassive bilateral pulmonary emboli with acute right ventricular dysfunction and a coexisting patent foramen ovale with right-to-left shunt. He remained profoundly hypoxemic despite catheter-directed thrombolysis. He underwent surgical embolectomy with partial endarterectomy, resulting in clinical improvement. DISCUSSION: The management of acute submassive pulmonary embolism is undertaken on an individualized basis because of the wide spectrum of clinical presentations. In this report we review the literature and discuss the evidence behind the management of cases of acute pulmonary embolism complicated by hypoxemia from a patent foramen ovale. In a case of acute pulmonary embolism complicated by refractory hypoxemia from an intracardiac shunt, adjunctive therapies in addition to anticoagulation and thrombolysis must be considered.