Pulmonary Artery Denervation for Refractory Sjögren's Syndrome-Associated Pulmonary Arterial Hypertension: A Case Report of Combined Hemodynamic and Renal Recovery.

A 31-year-old woman with primary Sjögren's syndrome presented with severe pre-capillary pulmonary arterial hypertension (mean pulmonary arterial pressure 51 mmHg, pulmonary vascular resistance 14.7 Wood units) and active renal impairment (24-hour urinary protein 1290 mg). Despite initial immunosuppressive therapy and dual oral PAH-targeted treatment with ambrisentan and tadalafil, she remained at high risk. She subsequently underwent pulmonary artery denervation (PADN) using a 45-mm ablation catheter with 120-second radiofrequency applications at three sites on the main pulmonary artery. At 2-month follow-up, she demonstrated significant symptomatic improvement to WHO functional class I, with a 6-minute walk distance of 320 meters, improved hemodynamics (mean pulmonary arterial pressure 35 mmHg, pulmonary vascular resistance 5.63 Wood units), and a reduced NT-proBNP level of 320 pg/mL, achieving an intermediate-low risk status according to the 2022 ESC/ERS guidelines. Renal function also improved (24-hour urinary protein 158 mg). This case suggests that PADN may be an effective adjunctive interventional strategy for refractory autoimmune PAH, even with concurrent renal involvement, facilitating simultaneous cardiopulmonary and renal recovery.