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 echocardiographi

SUBMITTER: Gregorio C 

PROVIDER: S-EPMC12462150 | biostudies-literature | 2025 Aug

REPOSITORIES: biostudies-literature