2022
DOI: 10.1002/pul2.12117
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Clinical characteristics and outcomes in pulmonary manifestations of systemic sclerosis: Contribution from pulmonary hypertension and interstitial lung disease severity

Abstract: Patients with systemic sclerosis complicated by both pulmonary hypertension (SSc‐PH) and interstitial lung disease (SSc‐PH‐ILD) have poor prognosis compared to those with SSc‐PH or SSc‐ILD alone. Little is known of how ILD severity affects outcomes in those with SSc‐PH, or how PH severity affects outcomes in those with SSc‐ILD. Herein, we aimed to delineate clinical features of patients with SSc‐PH and SSc‐ILD and determine to what degree PH and ILD severity contribute to mortality in patients with SSc. We con… Show more

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Cited by 4 publications
(1 citation statement)
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“…In SSc, registries have approximated that up to 65% of patients have or will develop radiographic evidence of ILD in the course of their disease [11]. While only a minority of these cases will be clinically significant and progressive, patients with advanced SSc-ILD often develop concomitant PH consistent with Group 3 disease, which portends a poor prognosis [12].…”
Section: Pulmonary Hypertension-who Classificationmentioning
confidence: 99%
“…In SSc, registries have approximated that up to 65% of patients have or will develop radiographic evidence of ILD in the course of their disease [11]. While only a minority of these cases will be clinically significant and progressive, patients with advanced SSc-ILD often develop concomitant PH consistent with Group 3 disease, which portends a poor prognosis [12].…”
Section: Pulmonary Hypertension-who Classificationmentioning
confidence: 99%