Background Connective tissue disease (CTD) is a type of autoimmune disease that affects many organs and tissues. The most common manifestations in the lungs include interstitial lung disease, pulmonary arterial hypertension (PAH), pulmonary infarction, and pleuritis. Most CTD-PAH patients without symptoms or with mild PAH are overlooked by doctors and therefore cannot be diagnosed or treated early. In this study, we retrospectively analysed the clinical manifestations, laboratory features and therapeutic regimens of 111 CTD-PAH patients selected from 2584 CTD patients to improve our understanding of this disease.
Objectives This study aimed to estimate the incidence of pulmonary arterial hypertension (PAH) in connective tissue disease (CTD) patients, assess the relationship between the patients’ clinical manifestations and the type of PAH, and discuss the effects of different diseases and treatments on prognosis.
Methods A retrospective evaluation of 111 CTD-PAH patients from 2584 CTD patients was conducted. Clinical data and experimental indexes were collected from the patients at baseline and after 1,2,3,5 years of treatment to investigate the risk factors of PAH and to assess whether different treatments or primary diseases would make a difference in the prognosis.
Results CTD-PAH was most prevalent in SSc (18.86%) and MCTD (12.00%) patients compared with other CTD patients (P<0.01). Patients with mild or moderate PAH exhibited a much better response to treatment than patients with severe PAH. SSc patients had worse clinical manifestations and haemodynamic parameters than SLE patients at the baseline and after treatment. The mortality rate in CTD-PAH patients was significantly lower than in CTD patients without PAH (0.85% vs 6.31%, P <0.05). The 5-year survival rate for targeted PAH therapy group has become significantly higher than non-targeted PAH therapy group (0.91% vs 0.71%, P=0.028).
Conclusions Raynaud’s phenomenon, anti-U1RNP(+), and ACA-IgG(+) are suggestive of a higher risk of PAH in patients with CTD. The treatment outcomes in patients with severe PAH or patients with SSc and PAH were both poor. Targeted therapy can improve the 5-year survival rate of CTD-PAH patients.
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Disclosure of Interest None Declared