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Gadre, Shruti; Minai, Omar A.; Zhang, Qi; Budev, Marie; Tonelli, Adriano R.

doi:10.6002/ect.2016.0209

Cited by 6 publications

(5 citation statements)

References 45 publications

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“…All of the included articles were retrospective ones with the majority of them having been conducted at a single institution (single center: 13 [33][34][35][36][37][38][39][40][41][42][43][44][45], two centers: one [46], multicenter: three [47][48][49]) (Table 1). The US-based publications represented the majority of the collected data (11 out of 17).…”

Section: Resultsmentioning

confidence: 99%

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Systemic Sclerosis: Current State and Survival After Lung Transplantation

Minalyan¹,

Gabrielyan²,

Khanal³

et al. 2021

Cureus

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Systemic sclerosis (SSc) is an autoimmune disorder characterized by the involvement of skin and internal organs. With the introduction of angiotensin-converting enzyme inhibitors (ACEIs), scleroderma renal crisis (SRC) is no longer considered a leading cause of death in affected patients. In fact, pulmonary manifestations [interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH)] are currently the major cause of death in patients with SSc. Historically, many centers have been reluctant to offer lung transplantation to patients with SSc due to multiple extrapulmonary manifestations and the assumption of poor post-transplant survival. The purpose of this review is to highlight the recent advances in the evaluation and management of patients with pulmonary manifestations of SSc. We also engage in a systematic literature review to assess all the available data on the survival of patients with SSc after lung transplantation.

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Section: Resultsmentioning

confidence: 99%

“…Despite that, one-year survival was comparable between the two groups (100% for the SSc patients, 86.8% for the control group). After adjusting for multiple variables, the male gender and severe tricuspid regurgitation before lung transplantation were found to be independent predictors of poor post-LT survival [43].…”

Section: Survival Datamentioning

confidence: 95%

Systemic Sclerosis: Current State and Survival After Lung Transplantation

Minalyan¹,

Gabrielyan²,

Khanal³

et al. 2021

Cureus

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“…Lung transplantation is not contraindicated in patients with CTD-PAH. However, evaluating other potentially involved organs, especially the digestive system, kidneys, heart and skin, must be considered [15,26].…”

Section: Therapymentioning

confidence: 99%

“…Until now, it was thought that the treatment of arterial pulmonary hypertension in patients with PAH-CTD is less effective than in patients without CTD [11][12][13]. However, the latest data indicate that implementing PAH-CTD treatment at the initial stage of the disease (World Health Organization [WHO] functional class [FC] I or II) is associated with a better prognosis [14,15]. Thus, we present a review of the literature on the treatment and prognosis of patients with PAH-CTD.…”

Section: Introductionmentioning

confidence: 99%

Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases

Smukowska-Gorynia,

Gościniak,

Woźniak

et al. 2023

Preprint

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Pulmonary hypertension (PH) is a severe vascular complication of connective tissue diseases (CTD). Patients with CTD may develop PH belonging to different groups: 1) pulmonary arterial hypertension (PAH), 2) PH secondary to lung disease and/or hypoxia, 3) PH due to left heart disease, and 4) chronic thromboembolic pulmonary hypertension (CTEPH). PAH most often develops in systemic scleroderma (SSc), mostly in its limited variant. PAH-CTD is a progressive disease characterized by poor prognosis, therefore the early diagnosis should be established A specific treatment for PAH-CTD is currently available and recommended: prostacyclin derivative (treprostinil, epoprostenol, iloprost, selexipag), nitric oxide and natriuretic pathway: stimulators of soluble guanylate cyclase (sGC: riociguat) and phosphodiesterase 5 inhibitors (PDE5i: sildenafil, tadalafil), endothelin receptor antagonists (ERA: bosentan, macitentan, ambrisentan). Moreover, novel drugs e.g. sotatercept are intensively investigated in the clinical trials. The aim of this paper is to review the literature on the recent advances in treatment strategy and prognosis of patients with PAH-CTD.

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“…[ 76 ] CTD-associated PAH is not a contraindication for organ transplantation, and it has been shown that patients with SSc-associated PAH and IPAH have a comparable prognosis from organ transplantation. [ 77 ] However, as most patients with CTD-associated PAH are in a state of immunodeficiency due to the long-term use of glucocorticoids and immunosuppressants, the risk of opportunistic infection during perioperative periods of organ transplantation is significantly increased. Therefore, it is necessary to strengthen monitoring.…”

Section: Introductionmentioning

confidence: 99%

2020 Chinese expert-based consensus on the diagnosis and treatment of connective tissue disease associated pulmonary arterial hypertension

Wang

Zhang

et al. 2021

Rheumatology and Immunology Research

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Pulmonary arterial hypertension (PAH) is a severe complication of connective tissue disease (CTD) and is one of the leading causes of morbidity and mortality among patients with this condition. To establish an expert-based consensus on the diagnosis and treatment of CTD-associated PAH, a multidisciplinary consensus development panel was established. The consensus panel is composed of 45 experts in rheumatology, cardiology, pulmonology, and radiology, most of whom are members of the Group of Pulmonary Vascular and Interstitial Lung Diseases (ILD) Associated with Rheumatic Diseases. The consensus development panel compiled 9 recommendations for the diagnosis and treatment of CTD-associated PAH. It covers screening, diagnosis, disease evaluation, risk assessment, the use of immunosuppressive agents, and PAH-specific therapy with a treat-to-target approach. The consensus is intended to facilitate decision-making and standardize the care of CTD-associated PAH in China.

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Untitled

Cited by 6 publications

References 45 publications

Systemic Sclerosis: Current State and Survival After Lung Transplantation

Systemic Sclerosis: Current State and Survival After Lung Transplantation

Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases

2020 Chinese expert-based consensus on the diagnosis and treatment of connective tissue disease associated pulmonary arterial hypertension

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