Epidemiology and mortality of connective tissue disease-associated pulmonary arterial hypertension: A national cohort study in taiwan

Lin, Cheng‐Chieh; Ko, Chi Hua; Hsu, Chung Y.; Chen, Hung An

doi:10.1016/j.semarthrit.2020.06.005

Cited by 20 publications

(35 citation statements)

References 28 publications

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“…We could not completely account for the role of concomitant therapy which may have also contributed to measured effect sizes. Lastly, pulmonary hypertension is common and well-described in CTD-ILD [ 65 , 66 ]. Unfortunately, no included studies in our meta-analysis provided descriptions or assessments of pulmonary hypertension as assessed either by echocardiography or right heart catheterization.…”

Section: Discussionmentioning

confidence: 99%

Effect size of rituximab on pulmonary function in the treatment of connective-tissue disease-related interstitial lung disease: a systematic review and meta-analysis

et al. 2022

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Background Rituximab (RTX) has been previously reported as directed treatment in patients with connective-tissue disease-related interstitial lung diseases (CTD-ILD). A systematic assessment of treatment effect size on pulmonary function outcomes and related adverse effects in patients with CTD-ILD has not been previously reported. Methods We performed a systematic review and meta-analysis of published reports from PubMed, Embase, and Cochrane Libraries. Randomized and non-randomized controlled trials, case–control, cohort, and case series (with five or more cases) containing individual pulmonary function data and adverse effects were included. Study endpoints were pre- and post-treatment change in percent predicted forced vital capacity (FVC %) and diffusion capacity for carbon monoxide (DLCO%), along with reported drug-related adverse events. Results Twenty studies totaling 411 patients were identified with 14 included in the meta-analysis of pulmonary function and six in the descriptive review. Random effects meta-analysis of pre- and post-treatment pulmonary function findings demonstrated increases in FVC% (n = 296) (mean difference (MD) 4.57%, [95% CI 2.63–6.51]) and DLCO% (n = 246) (MD 5.0% [95% CI 2.71–7.29]) after RTX treatment. RTX treatment-related adverse effects were reported in 13.6% of the pooled cohort. Conclusions A systematic assessment of post-treatment effect size suggests a potential role for RTX in stabilizing or improving lung function in patients with CTD-ILD, with a modest but not insignificant adverse effect profile.

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Section: Discussionmentioning

confidence: 99%

Effect size of rituximab on pulmonary function in the treatment of connective-tissue disease-related interstitial lung disease: a systematic review and meta-analysis

et al. 2022

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“… 1 Due to the high prevalence of SLE in Asian nations, it has surpassed primary Sjogren’s syndrome (pSS) and systemic sclerosis (SSc) as the predominant cause of connective tissue disease (CTD)-associated PAH. 2 – 5 We recently published a study using a right heart catheterization (RHC)-based multicenter cohort to show the prognostic value of clinical and hemodynamic variables, including World Health Organization functional class (WHC FC), 6-min walking distance (6MWD), N-terminal pro-brain natriuretic peptide (NT-proBNP), and cardiac index (CI). 6 However, risk assessment and its predictive relevance in patients with SLE-associated PAH have not been thoroughly investigated.…”

Section: Introductionmentioning

confidence: 99%

Risk assessment in systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study

Wang

Qian

et al. 2022

Therapeutic Advances in Chronic Disease

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Objective: This study evaluated the prognostic value of the multivariable risk assessment for systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH). Methods: A multicenter prospective cohort of SLE-associated PAH (CSTAR-PAH cohort) diagnosed based on right heart catheterization (RHC) was established. Baseline and follow-up records were collected. Three methods of risk assessment, including (1) the number of low-risk criteria, based on World Health Organization functional class (WHO FC), 6-min walking distance (6MWD), right atrial pressure (RAP), and cardiac index (CI); (2) the three-strata stratification based on the average risk score of four variables (WHO FC, 6MWD, RAP, and CI); and (3) the four-strata stratification based on COMPARE 2.0 model were applied. A risk-assessment method using three noninvasive low-risk criteria was applied at the first follow-up visit. Survival curves between patients with different risk groups were compared by Kaplan–Meier’s estimation and log-rank test. Results: Three-hundred and ten patients were enrolled from 14 PAH centers. All methods of stratification at baseline and first follow-up significantly discriminated long-term survival. Survival rates were also significantly different based on the noninvasive risk assessment in first follow-up visit. Survival deteriorated with the escalation of risk from baseline to first follow-up. Patients with baseline serositis had a higher rate of risk improvement in their follow-up. Conclusion: The risk assessment has a significant prognostic value at both the baseline and first follow-up assessment of SLE-associated PAH. A noninvasive risk assessment can also be useful when RHC is not available during follow-up. Baseline serositis may be a predictor of good treatment response in patients with SLE-associated PAH.

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“…1 Pulmonary arterial hypertension (PAH) is a rare but severe complication of SLE, with incidence varying from 2.06% to 3.83% in affected patients. [2][3][4] Due to rapid…”

Section: Introductionmentioning

confidence: 99%

“…Advances in therapy and management over the past several decades have facilitated satisfaction of fertility needs of most women with SLE 1. Pulmonary arterial hypertension (PAH) is a rare but severe complication of SLE, with incidence varying from 2.06% to 3.83% in affected patients 2–4. Due to rapid disease progression and lack of effective treatments, the prognosis for patients with SLE-PAH is worse than for those with SLE alone, with a 3-year survival rate of 45.0%–89.4% 5–8…”

Section: Introductionmentioning

confidence: 99%

Impact of pregnancy in patients with systemic lupus erythematosus-associated pulmonary arterial hypertension: case series and literature review

Tian

Qian

et al. 2022

Lupus Sci Med

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ObjectiveThis study aimed to investigate the clinical characteristics and outcomes of pregnancy complicated by SLE-associated pulmonary arterial hypertension (SLE-PAH) in a case series and literature review.MethodsThis single-centre retrospective study included 10 consecutive pregnancies complicated by SLE-PAH confirmed by right heart catheterisation (RHC) at Peking Union Medical College Hospital between 2009 and 2020. A literature search was conducted and 14 pregnancy cases complicated by SLE-PAH were reviewed.ResultsAt the time of 10 patients’ initial visits, the average age was 30.00±5.72 years and the median disease duration of SLE and PAH was 34.5 (range 1–164) months and 2 (1–51) months. Two patients carried planned pregnancy, seven patients developed PAH during pregnancy and one pregnancy was unplanned. Further, nine patients had low disease activity, with Systemic Lupus Erythematosus Disease Activity Index between 0 and 4, and 30%, 30% and 40% of patients were of WHO functional class II, III and IV, respectively. All patients were evaluated by RHC and echocardiography. N-terminal pro-brain natriuretic peptide (NT-proBNP) levels were elevated in 70% of patients, with a median level of 776 (56–18 023) pg/mL. The median time of completed pregnancies in all patients was 31 (15–38) weeks and six patients delivered live infants. SLE activity and PAH severity improved in 70% of patients within 6 months after delivery. One patient died on the 15th day after induction of labour. In the remaining patients, all achieved a lupus low disease activity state; according to the European Society of Cardiology/European Respiratory Society risk stratification, seven were categorised at a lower risk state compared with their risk stratification during pregnancy, and two remained at intermediate risk. Additionally, 80% of patients exhibited mild impairments with WHO functional class I or II. The median NT-proBNP level was 184 (32–4003) pg/mL within 6 months after delivery. In the reviewed literature, the average age of patients was 30.09±5.37 years. The median time of completed pregnancies was 36 (28–40) weeks. More cases were planned and successful, and the survival rates of mothers and neonates were 85.71% and 92.86%, respectively.ConclusionsSuccessful pregnancy could be possible in women with SLE-PAH if SLE-PAH treatment goals are achieved under proper therapies, careful monitoring and thorough evaluations.

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Epidemiology and mortality of connective tissue disease-associated pulmonary arterial hypertension: A national cohort study in taiwan

Cited by 20 publications

References 28 publications

Effect size of rituximab on pulmonary function in the treatment of connective-tissue disease-related interstitial lung disease: a systematic review and meta-analysis

Effect size of rituximab on pulmonary function in the treatment of connective-tissue disease-related interstitial lung disease: a systematic review and meta-analysis

Risk assessment in systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study

Impact of pregnancy in patients with systemic lupus erythematosus-associated pulmonary arterial hypertension: case series and literature review

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