According to a press release from EULAR, immunosuppressive targeted therapies may reduce the risk of mortality and worsening of precapillary pulmonary hypertension over time in patients with systemic sclerosis. Experts explained that pulmonary hypertension may be postcapillary or precapillary, affecting 9% to 15% of patients with systemic sclerosis. Although immunosuppressants are currently the standard of care for those with skin or musculoskeletal manifestations, experts stressed that their effect on precapillary pulmonary hypertension is still unclear. In a new study—presented by Bruni et al at the EULAR 2023 Congress and simultaneously published in the Annals of the Rheumatic Diseases—investigators examined the data of 755 patients from the European Scleroderma Trials and Research (EUSTAR) registry to determine the impact of immunosuppressants and immunosuppressive targeted therapies on systemic sclerosis–associated precapillary pulmonary hypertension. At baseline, the investigators found that patients who were treated with immunosuppressants experienced higher rates of interstitial lung disease as well as diffuse skin, joint, and muscle involvement compared with those who did not receive immunosuppressants. After a follow-up of 3 years, the investigators showed that 70% of patients who were treated with immunosuppressants experienced a morbidity-mortality event, which they defined as the first event over time between death or worsening of precapillary pulmonary hypertension. However, immunosuppressive targeted therapies were associated with a reduced risk of morbidity-mortality events. The investigators concluded that treatment with tocilizumab and rituximab demonstrated signals for a risk reduction in mortality and worsening of precapillary pulmonary hypertension in this patient population.
June 23, 2023