Department of Medicine, State University of New York and Upstate Medical University, Syracuse, USA.
JAMA. 2011 Jan 26;305(4):391-9. doi: 10.1001/jama.2011.10.
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.
结节病是一种多系统肉芽肿性疾病，最常影响肺部，并可能导致严重的发病率。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、高钙血症和肾衰竭。大约三分之一的受影响者中，结节病持续为慢性疾病。关于疾病过程的临床陷阱和误解使这些人群面临护理延迟或不足的风险。虽然非干酪样肉芽肿是结节病的组织病理学标志，但它们也没有特异性。目前尚无针对结节病的诊断性检测方法，因此诊断仍然是排除性的。虽然结节病的病因仍不清楚，但最近对其免疫发病机制的深入了解使研究人员更接近于找到更有效的治疗方法。尽管皮质类固醇具有不良影响，但在需要治疗时，它们仍然是标准的治疗方法。针对涉及 CD4 型 1 辅助 T 细胞的机制的新型药物和生物制剂的临床研究可能提供更有效、耐受性更好的治疗方法。
