scholarly journals Review of the diagnosis and pharmacological management of pulmonary arterial hypertension in connective tissue disease

2016 ◽  
Vol 16 (1) ◽  
pp. 14-22
Author(s):  
Chun-Pong Tang ◽  
Ka-Lai Lee ◽  
King-Yee Ying
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Functional Status ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Term Survival ◽  
Pharmacological Management ◽  
Pulmonary Arterial

Abstract Connective-tissue-disease-associated pulmonary arterial hypertension (CTD-PAH) is a well-recognised pulmonary complication most commonly seen in patients with systemic sclerosis, followed by systemic lupus erythematosus and mixed connective tissue disease. In systemic-sclerosis-associated-pulmonary arterial hypertension (SSc-PAH), patients usually present late and the progression can be rapid with poor prognosis and survival. Early detection and prompt intervention of SSc-PAH is an important cornerstone to halt the disease progression. Various pulmonary vasodilatory agents were developed over the past two decades. They were shown to improve patients’ symptoms, functional status, exercise capacity, haemodynamics and long-term survival. Other immunosuppressive therapies also demonstrated to improve symptoms and functional status in certain group of patients. This article is to review the diagnosis and pharmacological management of patient with CTD-PAH.

Pulmonary arterial hypertension associated with connective tissue disease

ESC CardioMed ◽  
2018 ◽  
pp. 2531-2534
Author(s):  
Christopher P. Denton
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Heart Catheterization ◽  
Pulmonary Arterial ◽  
Detection And Diagnosis ◽  
Health Organization

Connective tissue disease-associated pulmonary arterial hypertension (PAH) falls within World Health Organization group 1. These patients are treated as others in this group, but there are important considerations regarding detection and diagnosis. Patients with connective tissue disease are at risk of PAH and should be screened with confirmation of diagnosis by right heart catheterization. Treatment follows the European Society of Cardiology guidelines for other forms of PAH. However, more information is available for systemic sclerosis PAH regarding screening, including the DETECT algorithm, and also in terms of long-term outcome of patients with borderline elevation of mean pulmonary arterial pressure. In cases of systemic lupus erythematosus or mixed connective tissue disease, immunosuppression should be given in conjunction with targeted PAH-specific therapy. Long-term outcomes for PAH in patients with connective tissue disease have improved since targeted specific therapies became available. Recent trials with morbidity–mortality endpoints and a high proportion of patients receiving combination treatment have shown comparable benefits for patients with connective tissue disease and PAH as for those with idiopathic PAH in contrast to the blunted response that was characteristic of earlier short-term studies assessing improvement in 6 min walk test distance.

scholarly journals Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients

2014 ◽  
Vol 44 (4) ◽  
pp. 963-972 ◽  
Author(s):  
Yan-Jie Hao ◽  
Xin Jiang ◽  
Wei Zhou ◽  
Yong Wang ◽  
Lan Gao ◽  
...  
Keyword(s):  
Risk Factors ◽  
Systemic Lupus Erythematosus ◽  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Chinese Patients ◽  
Systemic Lupus ◽  
Pulmonary Arterial

We sought to investigate the characteristics, survival and risk factors for mortality in Chinese patients with connective tissue disease (CTD)-associated pulmonary arterial hypertension (APAH) in modern therapy era.129 consecutive adult patients who visited one of three referral centres in China with a diagnosis of CTD-APAH confirmed by right heart catheterisation during the previous 5 years were enrolled. The end-point was all-cause death or data censoring.Systemic lupus erythematosus was the most common underlying CTD (49%) and systemic sclerosis just accounted for 6% in this cohort. The overall survival at 1 and 3 years was 92% and 80%, respectively. Pericardial effusion, a shorter 6-min walk distance, lower mixed venous oxygen saturation, higher pulmonary vascular resistance (PVR) and alkaline phosphatase (ALP), and lower total cholesterol levels were all associated with a higher risk of death among the study population. Higher PVR and ALP were independent predictors of mortality.In conclusion, unlike in western patients, systemic lupus erythematosus is the most common underlying disease in Chinese patients with CTD-APAH. The survival of Chinese patients with CTD-APAH in the modern treatment era is similar to that in western countries. Elevated PVR and ALP are independent risk factors for poor outcomes.

Idiopathic Pulmonary Arterial Hypertension

2010 ◽  
pp. 220-223
Author(s):  
Edward C. Rosenow
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Systemic Lupus ◽  
Presenting Symptoms ◽  
Progressive Dyspnea ◽  
Pulmonary Arterial

• 29-year-old woman • Progressive dyspnea on exertion • CXR findings were interpreted as normal • Pulmonary function was normal; diffusing capacity of lung for carbon monoxide was low-normal • Diagnosis was “neurotic” • In many patients with connective tissue disease, such as systemic lupus erythematosus (which this patient had), the presenting symptoms and findings are those of idiopathic pulmonary arterial hypertension. When the connective tissue disease becomes apparent, the pulmonary findings are then considered secondary pulmonary arterial hypertension...

scholarly journals Role of Osteopontin as a Potential Biomarker of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis and other Connective Tissue Diseases (CTDs)

2021 ◽  
Vol 14 (5) ◽  
pp. 394
Author(s):  
Mattia Bellan ◽  
Cristina Piccinino ◽  
Stelvio Tonello ◽  
Rosalba Minisini ◽  
Ailia Giubertoni ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Connective Tissue Disease ◽  
Connective Tissue Diseases ◽  
Cross Sectional ◽  
Potential Biomarker ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is a severe complication of connective tissue diseases (CTD). Its early diagnosis is essential to start effective treatment. In the present paper, we aimed to evaluate the role of plasma osteopontin (OPN) as a candidate biomarker of PAH in a cohort of CTD patients. OPN is a pleiotropic protein involved in inflammation and fibrogenesis and, therefore, potentially promising in this specific clinical context. We performed a cross-sectional observational study on a cohort of 113 CTD patients (females N = 101, 89.4%) affected by systemic sclerosis N = 88 (77.9%), mixed connective tissue disease N = 10 (8.8%), overlap syndrome N = 10 (8.8%) or undifferentiated connective tissue disease N = 5 (4.4%). CTD-PAH patients showed significantly higher OPN plasma values than patients with CTD alone (241.0 (188.8–387.2) vs. 200.7 (133.5–281.6) ng/mL; p = 0.03). Although OPN levels were directly correlated with age and inversely with glomerular filtration rate, they remained associated with PAH at multivariate analysis. In conclusion, OPN was significantly associated with PAH among patients with CTD, suggesting it may have a role as a non-invasive disease biomarker of PAH.

scholarly journals Replacing a phosphodiesterase-5 inhibitor with riociguat in patients with connective tissue disease-associated pulmonary arterial hypertension: a case series

2017 ◽  
Vol 7 (3) ◽  
pp. 741-746 ◽  
Author(s):  
Amresh Raina ◽  
Raymond L. Benza ◽  
Harrison W. Farber
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Clinical Response ◽  
Connective Tissue Disease ◽  
Case Series ◽  
Response To Treatment ◽  
Tolerability Profile ◽  
Phosphodiesterase Type 5 Inhibitors ◽  
Pulmonary Arterial

Patients with pulmonary arterial hypertension associated with connective tissue disease (PAH-PAH-CTD) such as systemic sclerosis (SSc) have a poorer response to treatment and increased mortality compared with patients with idiopathic PAH. Current treatment options for PAH-CTD include prostanoids, phosphodiesterase type-5 inhibitors (PDE-5i), endothelin receptor antagonists, and the soluble guanylate cyclase stimulator riociguat. In this case series, we describe three patients with PAH-CTD related to limited scleroderma who were switched from a PDE-5i to riociguat due to insufficient clinical response. The switch to riociguat was associated with an improvement in respiratory and hemodynamic parameters and a favorable tolerability profile. These cases demonstrate that switching to riociguat is a therapeutic option in patients with PAH-CTD who have not achieved a satisfactory clinical response to a PDE-5i.

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