Lupus-Associated Pulmonary Arterial Hypertension: A Success Story
Abstract
Background: Pulmonary arterial hypertension (PAH) is a severe and potentially life-threatening complication of systemic lupus erythematosus.
Case summary: 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.
Discussion and take-home messages: 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.
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Evidence Hub reads this as a case report, done in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health.
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