Survival in rheumatoid arthritis‐associated pulmonary arterial hypertension compared with idiopathic pulmonary arterial hypertension

Sadeghi, Saghar; Granton, John; Akhavan, Pooneh; Pasarikovski, Christopher R.; Roos, Adrienne M.; Thenganatt, John; Moric, Jakov

doi:10.1111/resp.12464

Cited by 17 publications

(9 citation statements)

References 35 publications

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“…The association between pulmonary hypertension and connective tissue diseases has long been recognised [35], and supervening pulmonary hypertension is associated with a poor outcome across the range of CTDs [36–38]. It would therefore be logical to assume that the mortality signal associated with PVV reflected a new imaging marker of pulmonary hypertension.…”

Section: Discussionmentioning

confidence: 99%

Evaluation of computer-based computer tomography stratification against outcome models in connective tissue disease-related interstitial lung disease: a patient outcome study

Jacob

Bartholmai

Rajagopalan

et al. 2016

BMC Med

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BackgroundTo evaluate computer-based computer tomography (CT) analysis (CALIPER) against visual CT scoring and pulmonary function tests (PFTs) when predicting mortality in patients with connective tissue disease-related interstitial lung disease (CTD-ILD). To identify outcome differences between distinct CTD-ILD groups derived following automated stratification of CALIPER variables.MethodsA total of 203 consecutive patients with assorted CTD-ILDs had CT parenchymal patterns evaluated by CALIPER and visual CT scoring: honeycombing, reticular pattern, ground glass opacities, pulmonary vessel volume, emphysema, and traction bronchiectasis. CT scores were evaluated against pulmonary function tests: forced vital capacity, diffusing capacity for carbon monoxide, carbon monoxide transfer coefficient, and composite physiologic index for mortality analysis. Automated stratification of CALIPER-CT variables was evaluated in place of and alongside forced vital capacity and diffusing capacity for carbon monoxide in the ILD gender, age physiology (ILD-GAP) model using receiver operating characteristic curve analysis.ResultsCox regression analyses identified four independent predictors of mortality: patient age (P < 0.0001), smoking history (P = 0.0003), carbon monoxide transfer coefficient (P = 0.003), and pulmonary vessel volume (P < 0.0001). Automated stratification of CALIPER variables identified three morphologically distinct groups which were stronger predictors of mortality than all CT and functional indices. The Stratified-CT model substituted automated stratified groups for functional indices in the ILD-GAP model and maintained model strength (area under curve (AUC) = 0.74, P < 0.0001), ILD-GAP (AUC = 0.72, P < 0.0001). Combining automated stratified groups with the ILD-GAP model (stratified CT-GAP model) strengthened predictions of 1- and 2-year mortality: ILD-GAP (AUC = 0.87 and 0.86, respectively); stratified CT-GAP (AUC = 0.89 and 0.88, respectively).ConclusionsCALIPER-derived pulmonary vessel volume is an independent predictor of mortality across all CTD-ILD patients. Furthermore, automated stratification of CALIPER CT variables represents a novel method of prognostication at least as robust as PFTs in CTD-ILD patients.Electronic supplementary materialThe online version of this article (doi:10.1186/s12916-016-0739-7) contains supplementary material, which is available to authorized users.

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Section: Discussionmentioning

confidence: 99%

Evaluation of computer-based computer tomography stratification against outcome models in connective tissue disease-related interstitial lung disease: a patient outcome study

Jacob

Bartholmai

Rajagopalan

et al. 2016

BMC Med

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“…The estimated survival rates at 1 year and 3 years were 84 and 78%, respectively, in our 20 RA patients with precapillary PH, as compared to a 1-year survival rate of 93% in a Canadian cohort [37]. These survival rates are certainly biased due to the retrospective design of the analysis, with an overrepresentation of prevalent cases of PH [38].…”

Section: Discussionmentioning

confidence: 99%

Association between Rheumatoid Arthritis and Pulmonary Hypertension: Data from the French Pulmonary Hypertension Registry

et al. 2018

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Background: Precapillary pulmonary hypertension (PH), and particularly pulmonary arterial hypertension (PAH), is a life-threatening complication of connective tissue diseases (systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease). The relationship between PH and rheumatoid arthritis (RA) has not been clearly established. Objectives: The aim of the study was to evaluate the relationship between precapillary PH and RA. Methods: We identified patients with PH and suspected RA included in the French PH Registry between 1 May 2004 and 31 December 2012 and evaluated the prevalence of confirmed RA in this population. RA phenotypes, clinical, functional, and hemodynamic data, and patient outcomes were recorded. Results: RA was confirmed in 20 patients (70% female; mean age 52 years) with precapillary PH, including 10 patients with PAH, 6 with severe PH due to lung disease, and 4 with chronic thromboembolic PH. The prevalence of RA was 0.35% (95% CI: 0.23–0.54) in the French PH Registry and 0.58% (95% CI: 0.30–1.11) in idiopathic PAH, comparable to that in the general population. The RA phenotype was characterized by the presence of specific RA autoantibodies and joint erosions in 75% of the patients. The outcomes of PH in the RA patients were unremarkable compared to those in other patients from the registry, and RA therapies had no major impact on the cardiopulmonary parameters. Conclusion: When precapillary PH occurs in RA patients, all PH subsets may be identified. The RA prevalence in the French PH Registry is similar to that in the general population, which does not support a specific association or an indication for PH screening in RA patients.

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“…However, guidelines only offer a general recommendation for PAH patients, there are no specific criteria for subgroups. In addition, most studies focused on iPAH and others, risk assessment for PAH‐CHD still needs further investigations …”

Section: Introductionmentioning

confidence: 99%

Guideline implementation and early risk assessment in pulmonary arterial hypertension associated with congenital heart disease: A retrospective cohort study

Deng

Jin

et al. 2019

Clinical Respiratory J

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IntroductionCurrent guidelines emphasize that accurate risk stratification is important for patients with pulmonary arterial hypertension (PAH), however, few suggestions have been specified for PAH associated with congenital heart disease (PAH‐CHD).ObjectivesThe aim of this study was to propose an accurate and simple system based on current guidelines for risk stratification in PAH‐CHD patients during 12‐month follow‐up.MethodsWe reviewed 288 Chinese PAH‐CHD patients between January 2014 and December 2016 in this retrospective cohort study. The low‐risk criteria according to 2015 European Society of Cardiology guidelines and the adverse events (AEs) during follow‐up were collected. The association between low‐risk criteria and AEs was assessed with Cox regression, and a simplified risk stratification system was proposed.ResultsThere were 105 PAH‐CHD patients included in the final analysis. Twenty‐nine patients had AEs defined as death, initiation of new or combined medication treatment, or re‐hospitalisation because of the PAH worsening. Among the low‐risk criteria, WHO/NYHA functional class, 6‐minute walking distance (6MWD), NT‐proBNP and SvO2 were significantly different between AE and AE‐free groups. However, 6MWD (HR = 0.08, 95% CI: 0.03‐0.19, P < 0.001) and NT‐proBNP (HR = 0.35, 95% CI: 0.16‐0.78, P = 0.01) were the only independent predictors of AEs in multivariable model. When taking them into a simplified system for risk stratification, the number of low‐risk criteria at diagnosis discriminated the risk of AEs (P < 0.001).ConclusionsAmong the low‐risk criteria proposed by current guidelines, 6MWD and NT‐proBNP predicted AEs independently for PAH‐CHD patients. Simplified risk stratification system by taking these two parameters numerically provides accurate prognostic information in PAH‐CHD patients.

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Survival in rheumatoid arthritis‐associated pulmonary arterial hypertension compared with idiopathic pulmonary arterial hypertension

Abstract: Compared with IPAH patients, RA-PAH patients have an older age of onset and lower baseline mPAP. RA-PAH patients have comparable survival to IPAH patients.

Cited by 17 publications

References 35 publications

Evaluation of computer-based computer tomography stratification against outcome models in connective tissue disease-related interstitial lung disease: a patient outcome study

Evaluation of computer-based computer tomography stratification against outcome models in connective tissue disease-related interstitial lung disease: a patient outcome study

Association between Rheumatoid Arthritis and Pulmonary Hypertension: Data from the French Pulmonary Hypertension Registry

Guideline implementation and early risk assessment in pulmonary arterial hypertension associated with congenital heart disease: A retrospective cohort study

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