结节病:主要疾病特征及管理进展。

IF 3.9 4区 医学 Q1 MEDICINE, GENERAL & INTERNAL
Clinical Medicine Pub Date : 2025-05-01 Epub Date: 2025-05-15 DOI:10.1016/j.clinme.2025.100326
Robina K Coker, Kathy M Cullen
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引用次数: 0

摘要

结节病是一种病因不明的复杂疾病,主要累及肺部,但可累及几乎任何器官。由于缺乏特异性标记物,诊断具有挑战性,需要评估临床特征,仔细成像,排除其他条件,理想情况下,组织活检显示非坏死性肉芽肿。超过90%的患者肺部受累,表现为干咳和呼吸困难等症状,以及发烧和体重减轻等全身症状。约30%的病例出现肺外表现,可影响任何器官,包括心脏、神经系统和眼睛。治疗涉及多学科方法,一些患者需要免疫抑制和抗纤维化治疗。尽管通常预后良好,但结节病可导致显著的发病率和死亡率,特别是肺部和心脏并发症。英夫利昔单抗(infliximab)和尼达尼布(nintedanib)等新兴疗法为难治性病例带来了希望,尽管它们存在严重感染和其他副作用的风险。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Sarcoidosis: Key disease aspects and update on management.

Sarcoidosis: Key disease aspects and update on management.

Sarcoidosis: Key disease aspects and update on management.

Sarcoidosis: Key disease aspects and update on management.

Sarcoidosis is a complex disease of unknown origin, primarily affecting the lungs but capable of involving almost any organ. Diagnosis is challenging due to the lack of specific markers and requires assessment of clinical features, careful imaging, exclusion of other conditions and, ideally, a tissue biopsy demonstrating non-necrotising granulomas. Over 90% of patients have pulmonary involvement, presenting with symptoms like dry cough and breathlessness, along with systemic signs such as fever and weight loss. Extrapulmonary manifestations occur in about 30% of cases and can affect any organ, including the heart, nervous system and eyes. Management involves a multidisciplinary approach with some patients requiring immunosuppressive and antifibrotic therapies. Despite generally good outcomes, sarcoidosis can lead to significant morbidity and mortality, particularly from pulmonary and cardiac complications. Emerging treatments like infliximab and nintedanib offer hope for refractory cases, although they carry risks of serious infections and other side effects.

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来源期刊
Clinical Medicine
Clinical Medicine 医学-医学:内科
CiteScore
7.20
自引率
0.00%
发文量
0
审稿时长
6-12 weeks
期刊介绍: Clinical Medicine is aimed at practising physicians in the UK and overseas and has relevance to all those managing or working within the healthcare sector. Available in print and online, the journal seeks to encourage high standards of medical care by promoting good clinical practice through original research, review and comment. The journal also includes a dedicated continuing medical education (CME) section in each issue. This presents the latest advances in a chosen specialty, with self-assessment questions at the end of each topic enabling CPD accreditation to be acquired. ISSN: 1470-2118 E-ISSN: 1473-4893 Frequency: 6 issues per year
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