A comparison of the predictive accuracy of three screening models for pulmonary arterial hypertension in systemic sclerosis

Abstract

IntroductionThere is evidence that early screening for pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) improves outcomes. We compared the predictive accuracy of two recently published screening algorithms (DETECT 2013 and Australian Scleroderma Interest Group (ASIG) 2012) for SSc-associated PAH (SSc-PAH) with the commonly used European Society of Cardiology/European Respiratory Society (ESC/ERS 2009) guidelines.MethodsWe included 73 consecutive SSc patients with suspected PAH undergoing right heart catheterization (RHC). The three screening models were applied to each patient. For each model, contingency table analysis was used to determine sensitivity, specificity, and positive (PPV) and negative (NPV) predictive values for PAH. These properties were also evaluated in an ‘alternate scenario analysis’ in which the prevalence of PAH was set at 10%.ResultsRHC revealed PAH in 27 (36.9%) patients. DETECT and ASIG algorithms performed equally in predicting PAH with sensitivity and NPV of 100%. The ESC/ERS guidelines had sensitivity of 96.3% and NPV of only 91%, missing one case of PAH; these guidelines could not be applied to three patients who had absent tricuspid regurgitant (TR) jet. The ASIG algorithm had the highest specificity (54.5%). With PAH prevalence set at 10%, the NPV of the models was unchanged, but the PPV dropped to less than 20%.ConclusionsIn this cohort, the DETECT and ASIG algorithms out-perform the ESC/ERS guidelines, detecting all patients with PAH. The ESC/ERS guidelines have limitations in the absence of a TR jet. Ultimately, the choice of SSc-PAH screening algorithm will also depend on cost and ease of application.Electronic supplementary materialThe online version of this article (doi:10.1186/s13075-015-0517-5) contains supplementary material, which is available to authorized users.

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

“…Consistent with the international literature, cardiopulmonary manifestations are the leading causes of SSc death in Australia, having superseded SRC in the last two decades . Screening for these complications is recommended and has been shown to improve both survival and HRQoL .…”

“…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 . As SSc progresses, patients experience functional disability, disfigurement, pain and psychological consequences, including depression, helplessness and diminished health‐related quality of life (HRQoL) …”

Prospects for improving outcomes in systemic sclerosis‐related pulmonary hypertension

“…With this algorithm, the number of missed PAH cases was 4%, compared with 29% with the TTE‐based approach recommended in the current ESC/ERS guidelines. Both the ASIG and DETECT algorithms were comparable in performance and outperformed the previous ESC/ERS TTE‐based recommendations for SSc‐PAH screening, suggesting that multimodal assessment tools are necessary for screening, and that the use of TTE can be restricted to those at high risk of PH …”

Cost savings with a novel algorithm for early detection of systemic sclerosis‐related pulmonary arterial hypertension: alternative scenario analyses

“…NT‐proBNP is released by cardiac myocytes under stress and has been shown to correlate with New York Heart Association functional class, exercise capacity and haemodynamics in SSc‐PAH . The new algorithm has been shown to be equally, if not more, sensitive and specific than the current guidelines with a negative predictive value (NPV) of 100% …”