Lupus-Associated Pulmonary Arterial Hypertension: A Success Story

Pulmonary arterial hypertension (PAH) is a severe and potentially life-threatening complication of systemic lupus erythematosus.

We present the case of a young woman with dyspnea at rest, hospitalized with a suspected systemic lupus erythematosus flare. Right heart catheterization confirmed pre-capillary pulmonary hypertension with a low cardiac index. She was classified as World Health Organization functional class IV. Instead of initiating triple therapy with a parenteral prostacyclin analog, we opted for immunosuppressive therapy combined with dual oral PAH-specific therapy. Seven days after admission, the patient improved to World Health Organization functional class II, with no clinical signs of heart failure and normalization of echocardiographic parameters. At 6 months, she remains asymptomatic, with hemodynamic reassessment confirming the absence of PAH criteria.

Early recognition of severe PAH as a cause of dyspnea and hemodynamic instability in rheumatologic patients is essential for timely intervention. Immunosuppressive therapy should be considered an integral component of PAH-specific treatment, as it may significantly improve prognosis in autoimmune-related PAH.