Chest
Original ResearchPulmonary Vascular DiseaseSurvival in Systemic Sclerosis With Pulmonary Arterial Hypertension Has Not Improved in the Modern Era
Section snippets
Study Patients
Patients registered in the University of Michigan pulmonary hypertension database from April 1996 to October 2010 were included (Institutional Review Board [HUM 41444]). The total study period was chosen to be inclusive of the epoprostenol option. The “modern era” was used to be consistent with Condliffe et al11 and Humbert et al13 as corresponding to the availability of oral PAH-specific therapies beginning in 2002 (endothelin receptor antagonists [ETas] and phosphodiesterase-5 inhibitors
Results
The characteristics of patients with SSc-PAH (n = 83) and PAHifa (n = 120) (IPAH, 107 [89%]; FPAH, seven [6%]; anorexigen, six [5%]) at the time of first visit are summarized in Table 1. About 80% were women in both groups. SSc was characterized as limited scleroderma in 79%, diffuse scleroderma in 18%, and sine scleroderma in 2%. The mean time from onset of non-Raynaud SSc symptoms to the RHC was 10.4 ± 7.1 years. Antinuclear antibody characteristics were available in 73 of the 83 patients
Discussion
In this single-center study with a median follow-up of 35 months (IQR, 18-75 months), patients presenting in the “modern era” with SSc-PAH had an 84% 1-, 60% 3-, and 51% 5-year survival, which did not differ from pre-2002, when the sole PH-specific treatment was epoprostenol. SSc-PAH had a greater than twofold increase in mortality when compared with PAHifa, as demonstrated by the survival curves in Figure 1. After adjusting for age, sex, and hemodynamic risk factors, SSc-PAH was associated
Conclusions
Despite the relatively somber results of our study and in the literature, there is evidence that an early detection strategy may improve outcome in SSc-PAH. When compared with a small cohort with incident SSc found in routine clinical practice and patients participating in the French PAH Registry, those in a systematic SSc PAH detection program had an impressive improvement in survival.37 Our data argue persuasively that passive approaches to recognition do not serve this population well.
Acknowledgments
Author contributions: Dr Rubenfire is the guarantor of the paper and takes responsibility for the integrity of the work as a whole, from inception to published article.
Dr Rubenfire: contributed to the study design, database, interpretation of data, drafting of the manuscript, and manuscript revision and review.
Dr Huffman: contributed to the abstraction of data from charts, database entry, interpretation of data, and drafting of the manuscript.
Ms Krishnan: contributed to the management of the
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2022, Cardiology ClinicsCitation Excerpt :Outcomes in patients with SSc with PAH are poor, particularly in comparison to other forms of PAH. Modern era cohort studies have reported 3-year survival ranging from 50% to 75%.30–33 The improved survival in the PHAROS registry may reflect inclusion of patients with less severe disease, as more than half of the subjects were in WHO functional class 1 or 2 at enrollment.
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2020, ChestCitation Excerpt :Second, for patients with FC III symptoms and low-risk hemodynamics, experts determined that selexipag could be considered for patients with IPAH+ irrespective of hospitalization in the last 6 months for PAH and other features, whereas for CTD-associated PAH, patients with no hospitalization in the last 6 months for PAH should have either abnormal RV function, abnormal BNP/NT-proBNP levels, or 6MWD ≤ 440 m. A possible explanation for the lower threshold for use in patients with FC II symptoms may be because of the poorer prognosis associated with scleroderma-associated PAH,16,17 the panelists perceived a need for more intensive therapy. However, the threshold for addition of selexipag appears to be more nuanced in patients with CTD-PAH vs IPAH+ with FC III symptoms and low-risk hemodynamics.
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2018, ChestCitation Excerpt :Though difficult to make direct comparisons to other cohorts, it is notable that the majority of PHAROS patients were classified as functional class I/II, whereas the majority of patients in cohorts with worse survival were functional class III/IV at the time of enrollment.6,10,18,25 Prior studies have identified functional class as an important predictor of survival.20,22,25,27 The recent Australian cohort used annual ECG and pulmonary function tests to screen for patients at high-risk for PAH.
Kynurenine pathway metabolism evolves with development of preclinical and scleroderma-associated pulmonary arterial hypertension
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Funding/Support: The authors have reported to CHEST that no funding was received for this study.
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