Sarcoidosis Clinical Presentation, Immunopathogenesis, and Therapeutics
Sarcoidosis Clinical Presentation, Immunopathogenesis, and Therapeutics
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DOI：
10.1001/jama.2011.10
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发表时间：
2011-01-26
影响因子：
120.7
通讯作者：
Fontana, Joseph R.
中科院分区：
文献类型：
--
作者：
Iannuzzi, Michael C.;Fontana, Joseph R.
结节病是一种多系统肉芽肿性疾病，最常影响肺部，可引起显著的发病率。结节病可表现为神经系统疾病、葡萄膜炎、失明、终末期肺纤维化、肺动脉高压、心律失常、心肌病、高钙血症和肾功能衰竭。结节病作为慢性疾病在大约三分之一的患者中持续存在。临床缺陷和对病程的误解使这一人群面临延误或护理不足的风险。虽然非干酪化肉芽肿是结节病的组织病理学标志，但它们也是非特异性的。没有结节病的病理诊断测试，因此诊断仍然是一种排除。虽然结节病的病因尚不清楚，但最近对其免疫发病机制的了解使研究人员更接近于找到更有效的治疗方法。当需要治疗时，糖皮质激素仍然是标准的护理，尽管它们有副作用。对涉及CD4 1型辅助性T细胞的新型药物和生物制剂靶向机制的临床研究可能会提供更有效、更耐受的治疗方法。《美国医学协会杂志》上。2011年;305 (4): 391 - 399 www.jama.com
Sarcoidosis is a multisystem granulomatous disorder that most often affects the lungs and may cause significant morbidity. Sarcoidosis can manifest as neurological disease, uveitis, blindness, end-stage pulmonary fibrosis, pulmonary hypertension, dysrhythmias, cardiomyopathy, hypercalcemia, and renal failure. Sarcoidosis persists as chronic disease in approximately one-third of those affected. Clinical pitfalls and misconceptions about the course of disease place this population at risk for delayed or inadequate care. While noncaseating granulomas are the histopathological hallmark of sarcoidosis, they also are nonspecific. No pathognomonic diagnostic test exists for sarcoidosis, so the diagnosis remains one of exclusion. While the etiology of sarcoidosis is still unknown, recent insights into its immunopathogenesis have moved investigators closer to finding more effective treatments. Corticosteroids remain the standard of care when treatment is indicated, despite their adverse effect profile. Clinical investigations of novel drugs and biological agents targeting mechanisms involving CD4 type 1 helper T cells may provide more effective, better tolerated therapies. JAMA. 2011;305(4):391-399 www.jama.com