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ERS 临床实践指南:结节病的治疗。

ERS clinical practice guidelines on treatment of sarcoidosis.

机构信息

Dept of Medicine, University of Cincinnati Medical Center, Cincinnati, OH, USA

INSERM UMR 1272, Université Sorbonne Paris Nord, AP-HP, Hôpital Avicenne, Bobigny, Groupe Hospitalier Paris-Saint Joseph, Paris, France.

出版信息

Eur Respir J. 2021 Dec 16;58(6). doi: 10.1183/13993003.04079-2020. Print 2021 Dec.

Abstract

BACKGROUND

The major reasons to treat sarcoidosis are to lower the morbidity and mortality risk or to improve quality of life (QoL). The indication for treatment varies depending on which manifestation is the cause of symptoms: lungs, heart, brain, skin or other manifestations. While glucocorticoids remain the first choice for initial treatment of symptomatic disease, prolonged use is associated with significant toxicity. Glucocorticoid-sparing alternatives are available. The presented treatment guidelines aim to provide guidance to physicians treating the very heterogenous sarcoidosis manifestations.

METHODS

A European Respiratory Society Task Force committee composed of clinicians, methodologists and patients with experience in sarcoidosis developed recommendations based on the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) methodology. The committee developed eight PICO (Patients, Intervention, Comparison, Outcomes) questions and these were used to make specific evidence-based recommendations.

RESULTS

The Task Force committee delivered 12 recommendations for seven PICOs. These included treatment of pulmonary, cutaneous, cardiac and neurologic disease as well as fatigue. One PICO question regarding small-fibre neuropathy had insufficient evidence to support a recommendation. In addition to the recommendations, the committee provided information on how they use alternative treatments, when there was insufficient evidence to support a recommendation.

CONCLUSIONS

There are many treatments available to treat sarcoidosis. Given the diverse nature of the disease, treatment decisions require an assessment of organ involvement, risk for significant morbidity, and impact on QoL of the disease and treatment.

摘要

背景

治疗结节病的主要原因是降低发病率和死亡率风险或提高生活质量(QoL)。治疗的指征取决于引起症状的表现:肺部、心脏、大脑、皮肤或其他表现。虽然糖皮质激素仍然是治疗有症状疾病的首选药物,但长期使用会导致明显的毒性。目前有糖皮质激素保留的替代药物。本治疗指南旨在为治疗非常异质性结节病表现的医生提供指导。

方法

由具有结节病经验的临床医生、方法学家和患者组成的欧洲呼吸学会(ERS)工作组委员会,根据 GRADE(推荐分级的评估、制定与评价)方法制定了建议。委员会制定了 8 个 PICO(患者、干预、比较、结局)问题,并使用这些问题制定了具体的基于证据的建议。

结果

工作组委员会为 7 个 PICO 中的 12 个建议提供了依据。这些建议包括治疗肺部、皮肤、心脏和神经系统疾病以及疲劳。一个关于小纤维神经病的 PICO 问题,由于证据不足,无法支持推荐。除了建议外,委员会还提供了关于如何在证据不足时使用替代治疗的信息。

结论

有许多治疗方法可用于治疗结节病。鉴于该病的多样性,治疗决策需要评估器官受累、发生严重发病率的风险以及疾病和治疗对生活质量的影响。

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