Early Mortality in a Multinational Systemic Sclerosis Inception Cohort

Abstract

Early mortality in SSc is substantial, and prevalent cohorts underestimate mortality in SSc by failing to capture early deaths, particularly in men and those with diffuse disease.

A systematic review of the epidemiology, disease characteristics and management of systemic sclerosis in Australian adults

“…Despite an improvement over the last three decades, morbidity and mortality in SSc remains high (Tables and ). Survival is well below age and gender matched controls, with an overall standardized mortality ratio (SMR) of 3.4 and a 10‐year survival of 84% in a newly diagnosed patient . Poor prognostic features include male gender, diffuse disease subtype (dcSSc), presence of PAH or SRC and the presence of Scl‐70 antibody .…”

“…Survival is well below age and gender matched controls, with an overall standardized mortality ratio (SMR) of 3.4 and a 10‐year survival of 84% in a newly diagnosed patient . Poor prognostic features include male gender, diffuse disease subtype (dcSSc), presence of PAH or SRC and the presence of Scl‐70 antibody . SSc is the predominant cause of death in most studies, accounting for 62.1% of deaths .…”

“…Another algorithm incorporates both asymmetric dimethylarginine (ADMA) and NT‐proBNP in detecting PAH . Predictors of SSc‐PAH include older age, female gender, lcSSc, ACA and higher functional class (II/III) . Predictors of mortality in SSc‐PAH include more severe PAH at diagnosis demonstrated by higher mean pulmonary arterial pressure, lower 6‐minute walk test, higher functional class and presence of pericardial effusion .…”

“…Despite an improvement over the last few decades, morbidity and mortality in SSc remain high with years of life lost (YLL) of up to 11.3 for women and 25.8 for men . Cardiopulmonary manifestations, namely pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD), remain the leading cause of SSc‐related death having superseded scleroderma renal crisis (SRC) in the last two decades .…”

Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension

“…This is highlighted in a recent large study showing an age and sex adjusted standardised mortality ratio (SMR) of 4.06 for newly diagnosed SSc patients, with 22.4 and 26.0 years of life lost (YLL) in women and men, respectively [2]. Cardiorespiratory manifestations, in particular pulmonary arterial hypertension (PAH), are the leading cause of SSc-related death [3].…”