Lupus-associated pulmonary hypertension: long-term response to vasoactive therapy

Area of Science:

• Cardiology
• Rheumatology
• Pulmonology

Background:

• Pulmonary hypertension (PH) is a severe complication of systemic lupus erythematosus (SLE).
• The efficacy of current vasoactive treatments for lupus-associated PH remains incompletely understood.

Purpose of the Study:

• To evaluate the effectiveness of pulmonary vasodilators in patients with lupus-associated PH.
• To assess the impact of vasoactive therapy on clinical and hemodynamic parameters in this patient cohort.

Main Methods:

• Retrospective analysis of 12 female patients diagnosed with lupus-associated PH.
• Patients received pulmonary vasodilator therapy, including epoprostenol, bosentan, treprostinil, and sildenafil, as monotherapy or combination therapy.
• Follow-up included assessment of six-minute walk distance (6MWD), functional class, and hemodynamic measurements.

Main Results:

• At baseline, patients presented with severe PH (mean pulmonary artery pressure 52 mmHg, median 6MWD 266m, functional class III-IV).
• After a mean follow-up of 41 months, significant improvements were observed: 6MWD increased by 139m (p=0.007), mean pulmonary artery pressure decreased by 18 mmHg (p=0.014), and cardiac index increased by 1.44 L/min/m(2) (p=0.016).
• Eight patients improved to functional class I-II, and no mortality or need for lung transplantation was reported. Therapy was well tolerated.

Conclusions:

• Vasoactive therapy is effective in achieving sustained clinical and hemodynamic improvements in patients with lupus-associated PH.
• Pulmonary vasodilators represent a viable treatment option for managing lupus-associated PH, improving patient outcomes and functional capacity.