Abstract

Abstract Background Pulmonary hypertension (PH) in adolescence is a devastating diagnosis with significant morbidity and mortality. Despite limited literature exists on treating PH with anti-inflammatory medications in humans, hereby we present the case of prednisolone induced clinical outcomes improvement in PH patient. Case Summary A 20-year-old woman presented with exertional dyspnea. Her past-two-years medical history was remarkable for tricuspid regurgitation with multiple readmission. Upon arrival, her saturation-75% and BP-110/60. She was in respiratory distress with JVP increased and high-pitched-holosystolic murmur. Laboratory studies was unremarkable. The x-ray revealed cardiomegaly and ECG showing RVH-RAE. Initial echocardiography demonstrated TR-Vmax 4.55m/s and PR-Vmax 3.62m/s. The RVSP estimated around 107mmHg, which was highly suggestive for PH. Due to the aggressive nature of the disease, her condition deteriorated despite management with IV-furosemide-(40mg/d), beta-blocker-(2.5mg/d), and sodium beraprost-(60µg/d). Following days, a 5-days pulse-dose-steroid (methylprednisolone) at 2mg/kg/day was administered. Her condition improved with saturation from 50% to 90% and significant reduced in dyspnea. The steroid was tapering down for another 7 days and she was discharged for further referral. Discussion Inflammation plays unequivocal role in the pathogenesis of PH, which was involved in the development of pulmonary vascular remodeling. The possible mechanism of prednisolone were related with the inhibition of platelet-derived growth factor (PDGF)-induced accelerated proliferation and migration of pulmonary artery smooth muscle cell, and prevented the activation of NF-κB, key transcription factor that controls inflammation, cell-proliferation, and apoptosis. Thus, given the ready availability and features of this medication compared with other PH-medication, we decided to utilize steroid in this patient.

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