Pulmonary arterial hypertension in idiopathic inflammatory myopathies

Sangès, S.; Yelnik, C.; Sitbon, Olivier; Benveniste, Olivier; Mariampillai, K.; Phillips-Houlbracq, Mathilde; Pison, Christophe; Deligny, C.; Inamo, Jocelyn; Cottin, Vincent; Mouthon, Luc; Launay, David; Lambert, Marc; Hatron, Pierre‐Yves; Rottat, Laurence; Humbert, Marc; Hachulla, É.

doi:10.1097/md.0000000000004911

Cited by 44 publications

(20 citation statements)

References 36 publications

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“…Autoimmune features in patients with ILD are important features in the natural history of the disease, and studies have reported that patients with inflammatory myopathies associated ILD have worse morbidity and higher mortality than patients without [ 47 ]. A retrospective cohort suggested that poorer pulmonary function test is more common in more severe PM/DM [ 48 ] and data from a nationwide prospective Pulmonary Hypertension Registry in France indicated a very possible association between inflammatory myopathies and pulmonary arterial hypertension [ 49 ]. This may, in part, explain the associations between high DRR with disease severity and mortality in the 1-year follow-up in our cohort.…”

Section: Discussionmentioning

confidence: 99%

AST/ALT ratio as a predictor of mortality and exacerbations of PM/DM-ILD in 1 year—a retrospective cohort study with 522 cases

Zhu

Wang

et al. 2020

Arthritis Res Ther

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Objective To assess the associations between aspartate transaminase/alanine transaminase ratio (DRR) and mortality in patients with polymyositis/dermatomyositis-associated interstitial lung disease (PM/DM-ILD). Patients and methods This was a retrospective cohort study, which included 522 patients with PM/DM-ILD whose DRR on admission were tested at West China Hospital of Sichuan University during the period from January 1, 2008, to December 31, 2018. Cox regression models were used to estimate hazard ratios for mortality in four predefined DRR strata (≤ 0.91, 0.91–1.26, 1.26–1.73, and > 1.73), after adjusting for age, sex, DRR stratum, diagnosis, overlap syndrome, hemoglobin, platelet count, white blood cell count, the percentage of neutrophils, neutrophil/lymphocyte ratio, albumin, creatine kinase, uric acid/creatinine ratio, triglycerides, or low-density lipoprotein. Results Higher DRR (> 1.73) was an independent predictor of 1-year mortality in multivariate Cox regression analysis (hazard ratio 3.423, 95% CI 1.481–7.911, p = .004). Patients with higher DRR more often required the use of mechanical ventilation and readmission for acute exacerbation of PM/DM-ILD at 1-year follow-up. Conclusion Higher DRR on admission for PM/DM-ILD patients are associated with increased mortality, risk of mechanical ventilation, and hospitalization in 1-year follow-up. This low-cost, easy-to-obtain, rapidly measured biomarker may be useful in the identification of high-risk PM/DM-ILD patients that could benefit from intensive management.

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Section: Discussionmentioning

confidence: 99%

AST/ALT ratio as a predictor of mortality and exacerbations of PM/DM-ILD in 1 year—a retrospective cohort study with 522 cases

Zhu

Wang

et al. 2020

Arthritis Res Ther

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“…Data on confirmed cases of CTEPH for the KB hospital were available from a prospective database set up for observational research ( tableau de bord du centre de référence hypertension artérielle pulmonaire et requêtes patients ). Confirmed cases from the second centre (LP) were extracted from the French PH registry, which was opened in 2002, and planned to enrol all consecutive patients aged ≥18 years with PH [14–16].…”

Section: Methodsmentioning

confidence: 99%

Identifying chronic thromboembolic pulmonary hypertension through the French national hospital discharge database

et al. 2019

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Chronic thromboembolic pulmonary hypertension (CTEPH), a rare pulmonary vascular disease, is often misdiagnosed due to nonspecific symptoms. The objective of the study was to develop, refine and validate a case ascertainment algorithm to identify CTEPH patients within the French exhaustive hospital discharge database (PMSI), and to use it to estimate the annual number of hospitalized patients with CTEPH in France in 2015, as a proxy for disease prevalence. As ICD-10 coding specifically for CTEPH was not available at the time of the study, a case ascertainment algorithm was developed in close collaboration with an expert committee, using a two-step process (refinement and validation), based on matched data from PMSI and hospital medical records from 2 centres. The best-performing algorithm (specificity 95%, sensitivity 70%) consisted of ≥1 pulmonary hypertension (PH) diagnosis during 2015 and any of the following criteria over 2009–2015: (i) CTEPH interventional procedure, (ii) admission for PH and pulmonary embolism (PE), (iii) PE followed by hospitalization in competence centre then in reference centre, (iv) history of PE and right heart catheterization. Patients with conditions suggestive of pulmonary arterial hypertension were excluded. A total of 3,138 patients hospitalized for CTEPH was estimated for 2015 (47 cases/million, range 43 to 50 cases/million). Assuming that patients are hospitalized at least once a year, the present study provides an estimate of the minimal prevalence of CTEPH and confirms the heavy burden of this disease.

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“…A majority of myositis-associated lung involvement manifests as ILD. Unlike in SSc, isolated PAH is rare in myositis [ 67 ]. ILD manifests early in the course of myositis, often found at the time of diagnosis [ 4 , 68 , 69 ].…”

Section: Interstitial Lung Disease (Ild)mentioning

confidence: 99%

Pharmacological Interventions for Pulmonary Involvement in Rheumatic Diseases

Kang

Song

2021

Pharmaceuticals

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Among the diverse forms of lung involvement, interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are two important conditions in patients with rheumatic diseases that are associated with significant morbidity and mortality. The management of ILD and PAH is challenging because the current treatment often provides only limited patient survival benefits. Such challenges derive from their common pathogenic mechanisms, where not only the inflammatory processes of immune cells but also the fibrotic and proliferative processes of nonimmune cells play critical roles in disease progression, making immunosuppressive therapy less effective. Recently, updated treatment strategies adopting targeted agents have been introduced with promising results in clinical trials for ILD ad PAH. This review discusses the epidemiologic features of ILD and PAH among patients with rheumatic diseases (rheumatoid arthritis, myositis, and systemic sclerosis) and the state-of-the-art treatment options, focusing on targeted agents including biologics, antifibrotic agents, and vasodilatory drugs.

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Pulmonary arterial hypertension in idiopathic inflammatory myopathies

Abstract: Supplemental Digital Content is available in the text

Cited by 44 publications

References 36 publications

AST/ALT ratio as a predictor of mortality and exacerbations of PM/DM-ILD in 1 year—a retrospective cohort study with 522 cases

AST/ALT ratio as a predictor of mortality and exacerbations of PM/DM-ILD in 1 year—a retrospective cohort study with 522 cases

Identifying chronic thromboembolic pulmonary hypertension through the French national hospital discharge database

Pharmacological Interventions for Pulmonary Involvement in Rheumatic Diseases

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