Diversity of hemodynamic types in connective tissue disease associated pulmonary hypertension: more than a subgroup of pulmonary arterial hypertension

Dong, Xingbei; Shi, Yue; Xia, Ying; Zhang, Xiao; Qian, Jianfei; Jiang, Peng; Wang, Qian; Weng, Li; Li, Mengtao; Du, Bin; Zeng, Xiaofeng

doi:10.1186/s12890-022-02081-0

Cited by 2 publications

(2 citation statements)

References 34 publications

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“…Dong et al found similar results among a group of 202 patients with different types of CTDs, of whom only approximately 10% had SSc. CTD patients with WHO Group 2 PH had worse short‐term survival but comparable 5‐year survival to those with CTD‐PAH 25 . WHO Group 3 PH due to ILD is also associated with poor outcomes.…”

Section: Is the Prognosis Of Ctd‐pah Different From Idiopathic Pah? I...mentioning

confidence: 92%

“…CTD patients with WHO Group 2 PH had worse short‐term survival but comparable 5‐year survival to those with CTD‐PAH. 25 WHO Group 3 PH due to ILD is also associated with poor outcomes. In fact, patients with PH associated with severe SSc‐ILD have a dismal 3‐year survival rate of 39%, which is worse than patients with SSc‐PAH without ILD.…”

Section: Is the Prognosis Of Ctd‐pah Different From Idiopathic Pah? I...mentioning

confidence: 99%

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Connective tissue disease‐associated pulmonary hypertension: A comprehensive review

Khangoora,

Bernstein,

King

et al. 2023

Pulm. circ.

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Connective tissue diseases (CTDs) can be associated with various forms of pulmonary hypertension, including pulmonary arterial hypertension (PAH), pulmonary veno‐occlusive disease, pulmonary venous hypertension, interstitial lung disease‐associated pulmonary hypertension, chronic thromboembolic pulmonary hypertension, and sometimes a combination of several processes. The prevalence of PAH varies among the different CTDs, with systemic sclerosis (SSc) having the highest at 8%–12%. The most recent European Society of Cardiology/European Respiratory Society guidelines recommend routine annual screening for PAH in SSc and CTDs with SSc features. As CTDs can be associated with a myriad of presentations of pulmonary hypertension, a thorough evaluation to include a right heart catheterization to clearly delineate the hemodynamic profile is essential in developing an appropriate treatment plan. Treatment strategies will depend on the predominant phenotype of pulmonary vasculopathy. In general, management approach to CTD‐PAH mirrors that of idiopathic PAH. Despite this, outcomes of CTD‐PAH are inferior to those of idiopathic PAH, with those of SSc‐PAH being particularly poor. Reasons for this may include extrapulmonary manifestations of CTDs, including renal disease and gastrointestinal involvement, concurrent interstitial lung disease, and differences in the innate response of the right ventricle to increased pulmonary vascular resistance. Early referral for lung transplant evaluation of patients with CTD‐PAH, particularly SSc‐PAH, is recommended. It is hoped that in the near future, additional therapies may be added to the armamentarium of effective treatments for CTD‐PAH. Ultimately, a better understanding of the pathogenesis of CTD‐PAH will be required to develop targeted therapies for this morbid condition.

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Section: Is the Prognosis Of Ctd‐pah Different From Idiopathic Pah? I...mentioning

confidence: 92%

Section: Is the Prognosis Of Ctd‐pah Different From Idiopathic Pah? I...mentioning

confidence: 99%

Connective tissue disease‐associated pulmonary hypertension: A comprehensive review

Khangoora,

Bernstein,

King

et al. 2023

Pulm. circ.

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Pulmonary Hypertension in the Course of Interstitial Lung Diseases—A Personalised Approach Is Needed to Identify a Dominant Cause and Provide an Effective Therapy

Kacprzak¹,

Tomkowski²,

Szturmowicz³

2023

Diagnostics

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The prevalence of pulmonary hypertension (PH) complicating interstitial lung diseases (ILDs) is 3.5–15% at an early stage, and up to 90% in ILD patients listed for lung transplantation. In addition, other types of PH may occur in patients with ILDs due to concomitant conditions. Therefore, any significant PH occurring in the setting of ILD requires a proper differential workup. PH increases morbidity and mortality in ILDs. The pathomechanisms underlying PH due to ILD (PH-ILD) are not fully known, and there is no straightforward correlation between the presence or severity of PH-ILD and the severity of ILD. Severe PH in mild ILD without other explanatory causes constitutes a dilemma of differentiating between PH due to ILD and pulmonary arterial hypertension coexisting with ILDs. The heterogeneity and poor prognosis of patients with ILDs coexisting with PH necessitate an individualised approach to the management of this condition. This review presents recent advances in understanding and treatment options in PH-ILD. It also addresses practical issues, such as when to suspect and how to screen for PH in ILD, what are the indications for right heart catheterisation, and how to approach an individual ILD patient to determine the dominant PH cause and apply adequate management.

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Diversity of hemodynamic types in connective tissue disease associated pulmonary hypertension: more than a subgroup of pulmonary arterial hypertension

Cited by 2 publications

References 34 publications

Connective tissue disease‐associated pulmonary hypertension: A comprehensive review

Connective tissue disease‐associated pulmonary hypertension: A comprehensive review

Pulmonary Hypertension in the Course of Interstitial Lung Diseases—A Personalised Approach Is Needed to Identify a Dominant Cause and Provide an Effective Therapy

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