Objective Patients with systemic sclerosis and both pulmonary hypertension and interstitial lung disease (SSc–PH‐ILD) generally carry a worse prognosis than patients with SSc and pulmonary arterial hypertension (SSc‐PAH) without ILD. There is no evidence of the efficacy of PAH therapies in SSc–PH‐ILD. We undertook this study to compare survival of and response to treatment in patients with SSc–PH‐ILD and those with SSc‐PAH. Methods We analyzed 128 patients (66 with SSc–PH‐ILD and 62 with SSc‐PAH) from 15 centers, in whom PH was diagnosed by right‐sided heart catheterization; they were prospectively included in the PH registry. All patients received PAH‐specific therapy. Computed tomography of the chest was used to confirm or exclude ILD. Results At baseline, patients with SSc–PH‐ILD had less severe hemodynamic impairment than those with SSc‐PAH (pulmonary vascular resistance 5.7 Wood units versus 8.7 Wood units; P = 0.0005) and lower diffusing capacity for carbon monoxide (median 25% [interquartile range (IQR) 18%, 35%] versus 40% [IQR 31%, 51%]; P = 0.0005). Additionally, patients with SSc–PH‐ILD had increased mortality (8.1% at 1 year, 21.2% at 2 years, and 41.5% at 3 years) compared to those with SSc‐PAH (4.1%, 8.7%, and 21.4%, respectively; P = 0.04). Upon treatment with PAH‐targeted therapy, no improvement in the 6‐minute walk distance was observed in either group. Improvement in the World Health Organization functional class was observed less frequently in patients with SSc–ILD‐PH compared to those with SSc‐PAH (13.6% versus 33.3%; P = 0.02). Hemodynamics improved similarly in both groups. Conclusion ILD confers a worse prognosis to SSc‐PH. Response to PAH‐specific therapy is clinically poor in SSc–PH‐ILD but was not found to be hemodynamically different from the response observed in SSc‐PAH.

Background: Concern for cardiovascular disease (particularly atrial fibrillation-AF) among women with breast cancer is becoming a major issue. We aimed at determining the incidence of cardiovascular disease events (AF, arterial and cardiac events, venous-thromboembolism-VTE) in patients diagnosed with breast cancer, and assessing potential risk factors. Methods: We reviewed medical records of all patients diagnosed with breast cancer from 2010 to 2011 in our cancer center. Baseline characteristics of patients and tumors were collected. The main outcome was the occurrence of cardiovascular disease events (AF, VTE, arterial and cardiac events) during the 5-years follow-up. Results: Among the 682 breast cancer patients, 22 (3.2%) patients had a history of atrial fibrillation. Thirty-four patients (5%) presented at least one cardiovascular disease event, leading to a cumulative incidence of 5.8% events at 5-years ([3.8-7.7] CI 95%), with most of them occurring in the first 2 years. AF cumulative incidence was 1.1% ([0.1-2.1] CI 95%). Factors associated with the occurrence of cardiovascular disease events (including AF) were an overexpression of HER-2 (HR 2.6 [1.21-5.56] p < 0.011), UICC-stage III tumors or more (HR 5.47 [2.78-10.76] p < 0.001) and pre-existing cardiovascular risk factors (HR 2.91 [1.36-6.23] p < 0.004). Conclusion: The incidence of cardiovascular disease events was 5.8% ([3.8-7.7] CI 95%), with HER-2 over-expression, UICC-stage III tumors or more and pre-existing cardiovascular diseases being associated with them. These findings call for the development of preventive strategies in patients diagnosed with breast cancer.

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