Lung vessel remodeling leads to increased pulmonary vascular resistance, causing pulmonary arterial hypertension (PAH), andconsequently right ventricular hypertrophy and failure. In patients suffering from systemic sclerosis (SSc), PAH can occur and is alife-threatening complication. Dysregulation of immune processes plays a crucial role in pulmonary vascular remodeling, as haspreviously been shown in Fos-related antigen-2 (Fra-2) transgenic (TG) mice, a model of SSc-PAH. Here, we investigate whethervascular remodeling in the Fra-2 TG model is driven by type 2 inflammation and is associated with vascular hyperresponsive-ness, an important feature of PAH pathobiology. Basal pulmonary arterial pressure and pulmonary vascular responsiveness tohypoxic ventilation and serotonin were increased in isolated, perfused, and ventilated lungs of Fra-2 TG mice compared withwild-type (WT) littermates. Similarly, contractile responses of isolated intrapulmonary arteries were elevated in Fra-2 TG mice. Wealso observed increased expression of contractile genes in Fra-2 overexpressing human pulmonary arterial smooth muscle cells(PASMCs) with elevated intracellular calcium levels after interleukin (IL)-13 stimulation. These findings were corroborated by tran-scriptomic data highlighting dysregulation of vascular smooth muscle cell contraction and type 2 inflammation in Fra-2 TG mice. In vivo, type 2-specific anti-inflammatory treatment with IL-13 neutralizing antibodies improved vascular remodeling in Fra-2 TGmice, similar to corticosteroid treatment with budesonide. Our results underscore the importance of type 2 inflammation and itspotential therapeutic value in PAH-associated pulmonary vascular remodeling and hyperresponsiveness in SSc-PAH. NEW & NOTEWORTHY In patients suffering from systemic sclerosis (SSc), pulmonary arterial hypertension (PAH) is a life-threat-ening complication linked to immune dysregulation. Preclinical analyses in Fos-related antigen-2 (Fra-2) transgenic (TG) mice, amodel of SSc-PAH, identify type 2 inflammation as a key driver of vascular remodeling. Anti-inflammatory treatment targetingtype 2 inflammation via IL-13 neutralizing antibodies improved pulmonary vascular remodeling. Thus, type 2-specific anti-inflam-matory treatment may be a promising therapeutic approach in SSc-PAH.