Mortality and Associated Factors in Patients with Systemic Sclerosis Associated Pulmonary Hypertension with and without Interstitial Lung Disease: A Long-Term Follow-up Study.

IF 1.8 4区 医学 Q3 RHEUMATOLOGY Modern Rheumatology Pub Date : 2024-10-17 DOI:10.1093/mr/roae095
Shirkhan Amikishiyev, Yasemin Yalçınkaya, Konul Mammadova, Numune Aliyeva, Gorkem Durak, Bahar Artim-Esen, Ahmet Gül, Ahmet Kaya Bilge, Gulfer Okumuş, Murat Inanc
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Abstract

Objectives: We aimed to analyze the prevalence, mortality, and prognostic factors in systemic sclerosis (SSc) patients with pulmonary hypertension (PH) with or without interstitial lung disease (ILD).

Methods: The associations between mortality and demographics, transthoracic echocardiography, right heart catheterization (RHC), pulmonary functional parameters at baseline, and treatment modalities in two groups; patients with pulmonary arterial hypertension (PAH, PH without significant ILD) and PH + ILD were evaluated.

Results: The mean age of the patients was 56.6±13.5 (range: 34-82, 44 women/ 2 men), and 23 (52.3%) were deceased during a median follow-up of 45 months. The survival rates of PH-SSc patients were 91% for the first year, 75% for 2 years, 68% for 3 years, and 43.1% for 5 years. The majority of deceased patients were in the PH + ILD group (p=0.007). The PH + ILD group had more diffuse skin involvement, anti-Scl-70 positivity, high C-reactive protein, low FVC, and lower DLCO values. The deceased patients had higher ePASP, low CO, and FVC values compared to surviving patients. Median survival time was significantly better in patients on combined therapy (44 vs. 61 months, p=0.01). The mortality-related factors in the PH + ILD group were decreased initial FVC, high ePASP on echocardiography, low cardiac output on RHC, deteriorated functional class, and monotherapy.

Conclusion: This is the first reported SSc-PH cohort from Turkey by a multidisciplinary team after the implementation of PAH-specific drugs. SSc-PH is a severe complication of SSc with high mortality especially in patients with accompanying severe ILD.

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伴有或不伴有间质性肺病的系统性硬化症相关肺动脉高压患者的死亡率及相关因素:长期随访研究
目的我们旨在分析伴有或不伴有间质性肺病(ILD)的肺动脉高压(PH)系统性硬化症(SSc)患者的患病率、死亡率和预后因素:方法:评估两组肺动脉高压(PAH,PH 无明显 ILD)患者和 PH + ILD 患者的死亡率与人口统计学、经胸超声心动图、右心导管检查(RHC)、基线肺功能参数和治疗方式之间的关系:患者的平均年龄为(56.6±13.5)岁(范围:34-82,44 名女性/2 名男性),在中位随访 45 个月期间,有 23 人(52.3%)死亡。PH-SSc患者第一年的存活率为91%,两年为75%,三年为68%,五年为43.1%。大部分死亡患者属于 PH + ILD 组(P=0.007)。PH + ILD组有更多弥漫性皮肤受累、抗Scl-70阳性、高C反应蛋白、低FVC和低DLCO值。与存活患者相比,死亡患者的ePASP、低CO和FVC值更高。接受联合治疗的患者中位生存时间明显更长(44 个月对 61 个月,P=0.01)。PH+ILD组的死亡相关因素包括初始FVC降低、超声心动图显示ePASP高、RHC显示心输出量低、功能分级恶化以及单一疗法:这是土耳其多学科团队在使用 PAH 专用药物后首次报告 SSc-PH 队列。SSc-PH是SSc的一种严重并发症,死亡率很高,尤其是伴有严重ILD的患者。
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来源期刊
Modern Rheumatology
Modern Rheumatology RHEUMATOLOGY-
CiteScore
4.90
自引率
9.10%
发文量
146
审稿时长
1.5 months
期刊介绍: Modern Rheumatology publishes original papers in English on research pertinent to rheumatology and associated areas such as pathology, physiology, clinical immunology, microbiology, biochemistry, experimental animal models, pharmacology, and orthopedic surgery. Occasional reviews of topics which may be of wide interest to the readership will be accepted. In addition, concise papers of special scientific importance that represent definitive and original studies will be considered. Modern Rheumatology is currently indexed in Science Citation Index Expanded (SciSearch), Journal Citation Reports/Science Edition, PubMed/Medline, SCOPUS, EMBASE, Chemical Abstracts Service (CAS), Google Scholar, EBSCO, CSA, Academic OneFile, Current Abstracts, Elsevier Biobase, Gale, Health Reference Center Academic, OCLC, SCImago, Summon by Serial Solutions
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