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2
2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Screening and Monitoring of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases.2023 年美国风湿病学会(ACR)/美国胸科学会(CHEST)系统性自身免疫性风湿病患者间质性肺病筛查和监测指南。
Arthritis Care Res (Hoboken). 2024 Aug;76(8):1070-1082. doi: 10.1002/acr.25347. Epub 2024 Jul 8.
3
Australian rheumatologists' perception of autologous haemopoietic stem cell transplantation for the treatment of systemic sclerosis: a cross-sectional survey.澳大利亚风湿病学家对自体造血干细胞移植治疗系统性硬化症的看法:一项横断面调查。
Intern Med J. 2024 Sep;54(9):1478-1482. doi: 10.1111/imj.16422. Epub 2024 May 31.
4
Therapeutic management of fibrosis in systemic sclerosis patients - an analysis from the Swiss EUSTAR cohort.系统性硬皮病患者纤维化的治疗管理——来自瑞士 EUSTAR 队列的分析。
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5
Exploratory clinical subgroup clustering in systemic sclerosis: Results from the Indian Progressive Systemic Sclerosis Registry.系统性硬化症的探索性临床亚组聚类:来自印度进行性系统性硬化症登记处的结果。
J Scleroderma Relat Disord. 2024 Feb;9(1):29-37. doi: 10.1177/23971983231215470. Epub 2023 Dec 14.
6
The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements.系统性硬化症中间质性肺疾病的识别与管理:基于证据的欧洲共识声明
Lancet Rheumatol. 2020 Feb;2(2):e71-e83. doi: 10.1016/S2665-9913(19)30144-4. Epub 2020 Jan 14.
7
Heterogeneity of determining disease severity, clinical course and outcomes in systemic sclerosis-associated interstitial lung disease: a systematic literature review.系统性硬皮病相关间质性肺病疾病严重程度、临床病程和结局的判断异质性:系统文献回顾。
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9
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10
2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension.2022年欧洲心脏病学会/欧洲呼吸学会肺动脉高压诊断和治疗指南。
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系统性硬化症的评估与处方趋势:印度风湿病学家的一项调查结果报告

Evaluation and prescription trends in systemic sclerosis: Report of a survey among Indian rheumatologists.

作者信息

Das Anna C, Sakthivel Yogananth, Shenoy Padmanabha, Sircar Geetabali, Mamadapur Mahabaleshwar, Buche Avinash Suresh, Shukla Dhaiwat, Bhojani Kaushik S, Nalawade Ajit, Goel Ruchika, Singh Yogesh Preet, Parimi Vijaya Prasanna, Sharma Aman, Agarwal Vikas, Khanna Dinesh, Kodali Ramya Sri, Janardana Ramya, Shobha Vineeta

机构信息

Department of Clinical Immunology and Rheumatology, St. John's Medical College Hospital, Bengaluru, India.

Department of Rheumatology, Centre for Arthritis and Rheumatism Excellence, Cochin, India.

出版信息

J Scleroderma Relat Disord. 2025 Apr 22:23971983251328797. doi: 10.1177/23971983251328797.

DOI:10.1177/23971983251328797
PMID:40290703
原文链接:
https://pmc.ncbi.nlm.nih.gov/articles/PMC12018362/
Abstract

OBJECTIVE

This study evaluates assessment and prescription trends in systemic sclerosis across different Indian healthcare settings, with a focus on diagnostic practices such as screening for interstitial lung disease, pulmonary hypertension, and adherence to recommended treatment protocols. The goal is to identify disparities and areas for improvement in the management of systemic sclerosis.

METHODS

A cross-sectional questionnaire-based survey was conducted among rheumatologists from teaching and non-teaching hospitals across India. Data collection focused on key diagnostic practices such as the modified Rodnan skin score, chest imaging, pulmonary function tests, and echocardiography. Organ-specific prescription trends were collected and compared between teaching and non-teaching centres.

RESULTS

The response rate for the survey was 70.5%. Teaching centres demonstrated higher adherence to performing modified Rodnan skin score at baseline (72.2%) compared to non-teaching hospitals (38.4%). For interstitial lung disease screening, overall, 93.7% performed chest imaging, with only 31.4% utilizing a High-Resolution CT thorax as the screening tool. Teaching centres performed 6MWT (79.5%) more often than non-teaching centres (64.7%). Echocardiography was commonly used for screening pulmonary hypertension (96.4%), while 16.5% reported using right heart catheterization. Steroids were used by 79.9% of participants at low doses (<10 mg) for a duration of less than 3 months, commonly for myositis(68%). Methotrexate(49.8%) and mycophenolate (38.3%) were the most prescribed first-line agents for systemic sclerosis-skin involvement. For systemic sclerosis-interstitial lung disease, mycophenolate (95%) was the most commonly used immunosuppression. Sequential addition of antifibrotic(62.4%) to immunosuppression was preferred over an upfront combination in systemic sclerosis-interstitial lung disease. The majority treated uncomplicated Raynaud's phenomenon with calcium channel blockers, followed by PDE5 inhibitors (61.4%). An upfront combination of endothelin receptor antagonists and PDE5i for systemic sclerosis-pulmonary hypertension was reported by 42.2%.

CONCLUSION

The study highlights differences in systemic sclerosis management trends among Indian rheumatologists. Despite variations in disease-encounter and practice settings, adherence to international recommendations in key domains and areas for further improvement are brought to light.

摘要

目的

本研究评估了印度不同医疗环境下系统性硬化症的评估和处方趋势,重点关注诸如间质性肺病筛查、肺动脉高压以及对推荐治疗方案的依从性等诊断实践。目标是识别系统性硬化症管理中的差异和改进领域。

方法

对印度各地教学医院和非教学医院的风湿病学家进行了一项基于问卷调查的横断面研究。数据收集集中在关键诊断实践上,如改良Rodnan皮肤评分、胸部成像、肺功能测试和超声心动图。收集并比较了教学中心和非教学中心特定器官的处方趋势。

结果

调查的回复率为70.5%。与非教学医院(38.4%)相比,教学中心在基线时进行改良Rodnan皮肤评分的依从性更高(72.2%)。总体而言,对于间质性肺病筛查,93.7%的人进行了胸部成像,只有31.4%的人使用高分辨率胸部CT作为筛查工具。教学中心进行6分钟步行试验(6MWT)的频率(79.5%)高于非教学中心(64.7%)。超声心动图常用于筛查肺动脉高压(96.4%),而16.5%的人报告使用右心导管检查。79.9%的参与者使用低剂量(<10毫克)类固醇,持续时间少于3个月,通常用于治疗肌炎(68%)。甲氨蝶呤(49.8%)和霉酚酸酯(38.3%)是系统性硬化症皮肤受累最常用的一线药物。对于系统性硬化症相关间质性肺病,霉酚酸酯(95%)是最常用的免疫抑制剂。在系统性硬化症相关间质性肺病中,与初始联合用药相比,在免疫抑制基础上序贯添加抗纤维化药物(62.4%)更受青睐。大多数人用钙通道阻滞剂治疗单纯性雷诺现象,其次是磷酸二酯酶5抑制剂(PDE5抑制剂,61.4%)。42.2%的人报告对系统性硬化症相关肺动脉高压采用内皮素受体拮抗剂和PDE5抑制剂的初始联合用药。

结论

该研究突出了印度风湿病学家在系统性硬化症管理趋势上的差异。尽管疾病诊治情况和实践环境存在差异,但关键领域对国际建议的依从性以及有待进一步改进的方面得以显现。