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Background and Aims

Pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) is a lethal complication affecting 8-15% of patients. Screening tests such as echocardiography and pulmonary function tests allow for triaging patients for diagnosis by right heart catheterization. Understanding risk factors of SSc-PAH could help differentiate high-risk patients. The aims were to determine risk factors of PAH in SSc.

Methods

A systematic review was conducted to determine associations with SSc-PAH, including clinical/disease characteristics, antibodies, labs and biomarkers. The frequencies of publications featuring each risk/association were reported.

Results

Among 2654 articles, 984 duplicates and 1578 irrelevant articles were removed, leaving 92 articles for manual screening. After excluding 55 papers with small sample sizes, publications from identical cohorts, not English language, or PAH not ascertained by RHC, 37 articles were eligible. A total of 43 factors for SSc-PAH were identified within seven categories. Several associations were due to PAH and risk factors such as dynpnea, right heart failure, and short 6-minute walk distance. Patient characteristics (14), pulmonary physiology (6), antibody profiles (6) and genetics/epigenetics (6) had the most numerous and diverse factors, while biomarkers (4) and other labs (2) features were infrequent. Low carbon monoxide (DLCO) (6), older age (4), longer disease duration (4), positive anticentromere antibodies (ACA) (4), telangiectasias (4), high brain natriuretic peptide (4) were frequent associations.

Conclusions

Risk factors for SSc-PAH such as ACA, older age, longer disease duration limited cutaneous SSc subset and presence of ILD may enrich screening programs. Genes and other antibody profiles are inconsistent and requires further validation.

Background and Aims

Inflammation plays a pivotal role in atherosclerosis, and the association between chronic inflammatory states and ischemic heart disease (IHD) was confirmed for several rheumatic diseases. Therefore, the constant state of inflammation to which sarcoidosis patients are exposed might pose as a risk factor for IHD.

The aim of this study is to assess the relation between sarcoidosis and IHD and its prognostic significance.

Methods

Based on data from Clalit Health Services, the largest healthcare organization in Israel, the medical records of 3,993 sarcoidosis patients and 19,856 controls were acquired. Controls were matched to sarcoidosis patients according to age and sex. Chi-square and student t-tests were used in order to compare variables distribution in the cohort. Variables associated with IHD were assessed by logistic regression model. Log-rank test was performed for survival analysis, while Cox proportional hazards method was utilised to evaluate variables related to increased risk of all-cause mortality.

Results

Sarcoidosis patients had a significantly higher proportion of IHD (856 cases, 21.4%) compared to controls (2999 cases, 15.1%, p < 0.001). A multivariate analysis demonstrated an association between sarcoidosis and IHD (adjusted OR 1.503, 95% CI 1.361–1.660). A 15-year follow-up revealed increased mortality among sarcoidosis patients: 710 (17.8%) deaths compared to 2121 (10.7%, p < 0.001). Sarcoidosis patients were also at increased risk for all-cause mortality compared to controls (multivariate model, adjusted HR 1.95, 95% CI 1.75–2.14).

Conclusions

Sarcoidosis is associated with greater risk for both ischemic hearth disease and all-cause mortality. Physicians should be alert of those risks when treating sarcoidosis patients.