In this thesis, several aspects of pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) are explored. A unique case study demonstrated the recovery of the nailfold capillary pattern in a patient with SSc following lung transplantation. Additionally, it was found that both interstitial lung disease (ILD) and PAH are linked to worsening microvascular structure. A study with 81 SSc patients showed that certain biomarkers, like Endostatin, can help differentiate those with PAH. Another analysis revealed that SSc-PAH patients had a distinct immune cell profile, with elevated levels of pro-inflammatory T cells. In a observational study, Selexipag, an oral medication, stabilized high-risk SSc-PAH patients' conditions. Further imaging studies showed that SSc-PAH patients had more cardiac fibrosis and inflammation compared to idiopathic PAH (IPAH) patients. Lastly, a literature review identified effective tools for measuring dyspnea in patients with ILD and PH.
Jacqueline Lemmers (1985) obtained her medical degree at Radboudumc in Nijmegen in 2012. In 2018, she temporarily interrupted her training as a medical specialist in the Department of Rheumatology to conduct PhD research in the same department. She is now in her final year as a rheumatology resident and expects to complete her training by the end of 2025.