Extract Pulmonary arterial hypertension (PAH) is a severe complication of connective tissue diseases (CTDs), including systemic sclerosis (SSc), systemic lupus erythematosus (SLE), primary Sjögren's syndrome (pSS), mixed CTD, and inflammatory myositis [1]. Patients with CTD-associated PAH exhibit significantly reduced long-term survival compared to those without PAH [2 - 6].
The refractory nature of underlying CTDs contributes to progressive clinical deterioration and poor outcomes. Current treatments beyond conventional immunosuppression and pulmonary vasodilators remain limited.
European Respiratory Journal published a clinical update in Critical Care on 12 Mar 2026.
The item focuses on CD19 CAR T-cell therapy for connective tissue disease-associated pulmonary arterial hypertension.
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