Risk factors associated with pulmonary arterial hypertension in patients with systemic sclerosis and implications for screening

ObjectivePulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis (SSc). In this longitudinal study, we aimed to identify factors associated with an unfavourable outcome in patients with SSc with early PAH (SSc-PAH) from the DETECT cohort.MethodsPatients with SSc-PAH enrolled in DETECT were observed for up to 3 years. Associations between cross-sectional variables and disease progression (defined as the occurrence of any of the following events: WHO Functional Class worsening, combination therapy for PAH, hospitalisation or death) were analysed by univariable logistic regression.ResultsOf 57 patients with PAH (median observation time 12.6 months), 25 (43.9%) had disease progression. The following factors (OR (95% CI)) were associated with disease progression: male gender (4.1 (1.2 to 14.1)), high forced vital capacity % predicted/carbon monoxide lung diffusion capacity (DLCO)% predicted ratio (3.6 (1.2 to 10.7)), high Borg Dyspnoea Index (1.7 (1.1 to 2.6)) and low DLCO% predicted (non-linear relationship).ConclusionMore than 40% of early-diagnosed patients with SSc-PAH had disease progression during a short follow-up time, with male gender, functional capacity and pulmonary function tests at PAH diagnosis being associated with progression. This suggests that even mild PAH should be considered a high-risk complication of SSc.

Download Full-text

Risk factors for pulmonary arterial hypertension in patients with systemic sclerosis

Nature Clinical Practice Rheumatology ◽

10.1038/ncprheum0148 ◽

2006 ◽

Vol 2 (5) ◽

pp. 236-236

Author(s):

Rachel Murphy

Keyword(s):

Risk Factors ◽

Systemic Sclerosis ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Pulmonary Arterial

Download Full-text

CHLD score, a new score based on traditional risk factor evaluation and long-term cardiovascular outcomes in patients with systemic sclerosis

Scientific Reports ◽

10.1038/s41598-021-99215-x ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Klaudia Gieszczyk-Strózik ◽

Maciej T. Wybraniec ◽

Małgorzata Widuchowska ◽

Ligia Brzezińska-Wcisło ◽

Przemysław Kotyla ◽

...

Keyword(s):

Diabetes Mellitus ◽

Risk Factors ◽

Chronic Kidney Disease ◽

Systemic Sclerosis ◽

Pulmonary Arterial Hypertension ◽

Kidney Disease ◽

Arterial Hypertension ◽

Maximal Velocity ◽

Pulmonary Arterial

AbstractThe aim of the study was to assess the predictors of major adverse cardiovascular events (MACE) in patients with systemic sclerosis (SSc) without pulmonary arterial hypertension. The study comprised 68 patients with SSc who were followed up for the median time of 99 (96; 107) months. The main exclusion criteria involved tricuspid regurgitation maximal velocity > 2.8 m/s and structural heart disease. At baseline the patients underwent clinical assessment of cardiovascular risk factors, 6-min walk test, transthoracic echocardiography and biomarker testing, including growth differentiation factor 15 (GDF-15). The primary composite endpoint was onset of MACE defined as death, myocardial infarction, myocardial revascularization and hospitalization for heart failure. The follow-up consisted of outpatient visits at 1 year intervals and telephone interview every 6 months. The baseline analysis revealed that chronic kidney disease (HR 28.13, 95%CI 4.84–163.38), lung fibrosis on high resolution computed tomography (HR 4.36, 95%CI 1.04–18.26) and GDF-15 concentration (unit HR 1.0006, 95%CI 1.0002–1.0010) were independent predictors of MACE occurrence. CHLD (Chronic kidney disease, Hypertension, hyperLipidaemia, Diabetes mellitus) score was formulated which assigned 1 point for the presence of arterial hypertension, hyperlipidaemia, diabetes mellitus and chronic kidney disease. After inclusion of CHLD score in Cox proportional model, it remained the only independent predictor of MACE onset (unit HR per 1 point 3.46; 95%CI 2.06–5.82, p < 0.0001). Joint assessment of traditional risk factors in the form of CHLD score may serve as a reliable predictor of long-term outcome in patients with SSc without pulmonary arterial hypertension.

Download Full-text