Survival in Systemic Sclerosis With Pulmonary Arterial Hypertension Has Not Improved in the Modern Era

Rubenfire, Melvyn; Huffman, Mark D.; Krishnan, Sangeetha; Seibold, James R.; Schiopu, Elena; McLaughlin, Vallerie V.

doi:10.1378/chest.12-0653

Cited by 72 publications

(49 citation statements)

References 37 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…A possible explanation for this finding could be that patients with diffuse SSc more frequently exhibit pulmonary and cardiac involvement which could result in obscuring of the electrical signal by electrophysiological changes not specifically related to pulmonary pressure elevation. Nonetheless, as previous studies show that especially patients with limited SSc are at greater risk of developing PH, the VG-RVPO has potential as screening marker in this specific patient population [5,7,27].…”

Section: Discussionmentioning

confidence: 96%

Electrocardiographic detection of pulmonary hypertension in patients with systemic sclerosis using the ventricular gradient

Couperus

Vliegen

Henkens

et al. 2016

Journal of Electrocardiology

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 96%

Electrocardiographic detection of pulmonary hypertension in patients with systemic sclerosis using the ventricular gradient

Couperus

Vliegen

Henkens

et al. 2016

Journal of Electrocardiology

View full text Add to dashboard Cite

“…5, 6 Based on these findings, Jais et al proposed a treatment algorithm for patients with P ulmonary arterial hypertension (PAH) is a refractory manifestations of connective tissue disease (CTD), even though a number of selective pulmonary vasodilators have become available. 1 Recent data from large registries of PAH patients have revealed that survival rates are better in patients with systemic lupus erythematosus (SLE) or mixed connective tissue disease (MCTD) than in those with systemic sclerosis (SSc). 2, 3 A possible reason for the better prognosis in patients with SLE-or MCTD-PAH is that immune and inflammatory mechanisms, rather than fibrotic processes, have a primary role in remodeling the pulmonary vasculature, resulting in favorable responses to immunosuppressive (IS) treatment.…”

mentioning

confidence: 99%

Predictors of Favorable Responses to Immunosuppressive Treatment in Pulmonary Arterial Hypertension Associated With Connective Tissue Disease

Yasuoka

Shirai

Tamura

et al. 2018

Circ J

View full text Add to dashboard Cite

Background:The potential efficacy of immunosuppressive (IS) treatment has been reported in patients with pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD), but its positioning in the treatment algorithm remains uncertain. The aim of this study was to identify predictors of favorable responses to first-line IS treatment. Methods and Results:This single-center retrospective study included 30 patients with PAH accompanied by systemic lupus erythematosus (SLE), mixed CTD (MCTD), or primary Sjögren's syndrome (SS) who received first-line IS treatment alone or in combination with pulmonary vasodilators. When short-term treatment response was defined as an improvement in World Health Organization functional class at 3 months, 16 patients (53%) were short-term responders. Simultaneous diagnosis of PAH and CTD, and the use of immunosuppressants, especially intravenous cyclophosphamide, in addition to glucocorticoids were identified as independent predictors of a short-term response (P=0.004 and 0.0002, respectively). Cumulative rates free of PAH-related death were better in short-term responders than non-responders (P=0.04), and were best in patients with a simultaneous diagnosis of PAH and CTD who were treated initially with a combination of glucocorticoids and immunosuppressants. Conclusions:Patients with a simultaneous diagnosis of PAH and CTD, including SLE, MCTD, and primary SS, should receive intensive IS treatment regimens to achieve better short-and long-term outcomes.

show abstract

“…Prior studies have examined whether targeted therapy for PAH improves morbidity in patients with CTD-PAH to the same extent as that seen in IPAH (8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20). These studies were limited by inadequate comparators, small sample sizes, the use of unvalidated outcomes, and (in the case of epidemiologic studies) the possibility of lead-time and length-time bias caused by screening for PAH in patients with SSc (21).…”

mentioning

confidence: 99%

Comparison of Treatment Response in Idiopathic and Connective Tissue Disease–associated Pulmonary Arterial Hypertension

Rhee

Gabler

Sangani

et al. 2015

Am J Respir Crit Care Med

View full text Add to dashboard Cite

Rationale: Studies suggest that patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) have a poorer treatment response to therapies for PAH compared with patients with idiopathic PAH (IPAH), but individual randomized controlled trials (RCTs) have been underpowered to examine differences within these subgroups.Objectives: To compare the effect of therapy for PAH in CTD-PAH versus IPAH.Methods: We obtained individual participant data from phase III placebo-controlled RCTs of therapies for PAH submitted to the U.S. Food and Drug Administration for drug approval. A treatment-bydiagnosis interaction term evaluated differences in treatment response between CTD-PAH and IPAH. Outcomes included change in 6-minute-walk distance (Δ6MWD) from baseline to 12 weeks, clinical worsening, and all-cause mortality.Measurements and Main Results: The study sample included 827 participants with CTD-PAH and 1,935 with IPAH from 11 RCTs. Patients with CTD-PAH had less improvement in 6MWD when assigned to active treatment versus placebo compared with patients with IPAH (difference in treatment effect on Δ6MWD in CTD-PAH vs. IPAH, 217.3 m; 90% confidence interval, 231.3 to 23.3; P for interaction = 0.043). Treatment was less effective in reducing the occurrence of clinical worsening in CTD-PAH versus IPAH (P for interaction = 0.012), but there was no difference in the placebo-adjusted effect of treatment on mortality (P for interaction = 0.65).Conclusions: Treatment for PAH was less effective in CTD-PAH compared with IPAH in terms of increasing 6MWD and preventing clinical worsening. The heterogeneity of treatment response supports the need for identifying therapies that are more effective for CTD-PAH.

show abstract

Survival in Systemic Sclerosis With Pulmonary Arterial Hypertension Has Not Improved in the Modern Era

Cited by 72 publications

References 37 publications

Electrocardiographic detection of pulmonary hypertension in patients with systemic sclerosis using the ventricular gradient

Electrocardiographic detection of pulmonary hypertension in patients with systemic sclerosis using the ventricular gradient

Predictors of Favorable Responses to Immunosuppressive Treatment in Pulmonary Arterial Hypertension Associated With Connective Tissue Disease

Comparison of Treatment Response in Idiopathic and Connective Tissue Disease–associated Pulmonary Arterial Hypertension

Contact Info

Product

Resources

About