Treatment of Pulmonary Hypertension in Patients with Connective Tissue Disease and Interstitial Lung Disease

Mittoo, Shikha; Jacob, Thomas; Craig, A.B.; Bshouty, Zoheir

doi:10.1155/2010/686098

Cited by 16 publications

(10 citation statements)

References 23 publications

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“…This study is the first to show possible survival benefits in patients with PH associated with CPFE, CTD‐IP, or IP when treated with PDE‐5I. One small study showed that treatment with bosentan and/or sildenafil conferred a small survival benefit in patients with PH associated with CTD‐IP . Subsequent analysis of the Sildenafil Trial of Exercise Performance in Indiopathic Pulmonary Fibrosis (STEP‐IPF) study revealed that in patients with IPF and right ventricular systolic dysfunction, sildenafil treatment preserved exercise capacity more than placebo control .…”

Section: Discussionmentioning

confidence: 90%

Multi‐institutional retrospective cohort study of patients with severe pulmonary hypertension associated with respiratory diseases

et al. 2015

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Background and objective: Pulmonary hypertension (PH) is often associated with respiratory diseases, but only a small number of patients present with severe PH defined as mean pulmonary arterial pressure ≥ 35 mm Hg. We here conducted a multicenter, retrospective study of patients with severe PH associated with respiratory diseases (R-PH) to reveal their demographics, treatment, prognosis and determinants of prognosis. Methods: From 101 patients with severe R-PH collected by postal survey at the first stage, 70 patients with four major diseases (chronic obstructive pulmonary disease (COPD), combined pulmonary fibrosis with emphysema (CPFE), interstitial pneumonia associated with connective tissue disease (CTD-IP), interstitial pneumonia (IP)) and normal pulmonary arterial wedge pressure were studied for clinical characteristics, treatment and prognosis.

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Section: Discussionmentioning

confidence: 90%

Multi‐institutional retrospective cohort study of patients with severe pulmonary hypertension associated with respiratory diseases

et al. 2015

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“…[115116] These studies have suggested potential benefit, but larger studies are needed to draw firm conclusions.…”

Section: Management: Pulmonary Arterial Hypertension Specificmentioning

confidence: 99%

Overview of Current Therapeutic Approaches for Pulmonary Hypertension

2011

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There have been tremendous strides in the management of pulmonary hypertension over the past 20 years with the introduction of targeted medical therapies and overall improvements in surgical treatment options and general supportive care. Furthermore, recent data shows that the survival of those with pulmonary arterial hypertension is improving. While there has been tremendous progress, much work remains to be done in improving the care of those with secondary forms of pulmonary hypertension, who constitute the majority of patients with this disorder, and in the optimal treatment approach in those with pulmonary arterial hypertension. This article will review general and targeted medical treatment, along with surgical interventions, of those with pulmonary hypertension.

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“…A retrospective study described treatment with PAH-specifi c therapies, bosentan and sildenafi l for 13 CTD-PAH-ILD patients, among which four had overlap syndromes. The survival estimate at a median duration of 34 months was 85% with mortality rates greater among patients with SSc versus other CTD subtypes [6].…”

Section: Discussionmentioning

confidence: 92%

“…Patients with SSc-PAH have a worse prognosis compared to patients with other forms of PAH with one-year survival rates ranging from 1%-50% [6]. Even when compared to patients with other forms of CTD-PAH, survival is markedly reduced in SSc-PAH.…”

Section: Discussionmentioning

confidence: 98%

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Interstitial Lung Disease and Pulmonary Arterial Hypertension in Overlap Syndrome: A Case Report

Frio¹,

Navarra²

2019

jmust

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OBJECTIVE To present the onset of severe pulmonary arterial hypertension (PAH) in a patient with interstitial lung disease (ILD) associated with overlap syndrome. CASE PRESENTATION A 42-year-old female was diagnosed with overlap syndrome consisting of systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and rheumatoid arthritis (RA). The serologic profi le included positive antinuclear antibody (ANA), anti-dsDNA, anti-RNP, anti-Ro, anti-Scl70, anti-Sm, rheumatoid factor and hypocomplementemia (C3, C4). She had chronic stable ILD for 17 years maintained on hydroxychloroquine (HCQ), prednisone 5 mg/day and indacaterol. The current admission was due to progressive dyspnea and right-sided heart failure over the past month. Chest radiograph showed pulmonary congestion, and 2-dimensional echocardiography (2DE) disclosed severe PAH with systolic pulmonary arterial pressure (SPAP) of 76 mmHg by tricuspid regurgitation (TR) jet, dilated right ventricle (RV) with poor systolic function, moderate pericardial effusion with no signs of tamponade. She received furosemide, beraprost, sildenafi l, and prednisone was increased to 20 mg/ day. Two weeks following discharge, there was complete resolution of symptoms and repeat 2DE showed non-dilated RV with good systolic function, normal SPAP of 21.4 mmHg and minimal pericardial effusion. Prednisone was tapered to 5 mg/day; beraprost, sildenafi l and HCQ were continued. CONCLUSION Overlap syndrome was diagnosed by the combination of clinical features and serology distinctive of SLE, SSc and RA. Her illness, particularly ILD, was adequately controlled over several years, until the recent onset of PAH complicated by right-sided heart failure. The dramatic response to high-dose steroids is more consistent with infl ammatory vasculitis of SLE activity rather than fi brosis typical of SSc.

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Treatment of Pulmonary Hypertension in Patients with Connective Tissue Disease and Interstitial Lung Disease

Abstract: Treatment using PH-specific therapies in patients with CTD, PH and ILD was well tolerated. Further studies to investigate the efficacy of PH-specific therapies in CTD-PH-ILD patients are warranted.

Cited by 16 publications

References 23 publications

Multi‐institutional retrospective cohort study of patients with severe pulmonary hypertension associated with respiratory diseases

Multi‐institutional retrospective cohort study of patients with severe pulmonary hypertension associated with respiratory diseases

Overview of Current Therapeutic Approaches for Pulmonary Hypertension

Interstitial Lung Disease and Pulmonary Arterial Hypertension in Overlap Syndrome: A Case Report

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