Department of Medicine, Karolinska University Hospital, Karolinska Institutet and Centre for Molecular Medicine, Stockholm, Sweden.
J Intern Med. 2014 Apr;275(4):335-49. doi: 10.1111/joim.12198. Epub 2014 Feb 5.
Sarcoidosis can be a major therapeutic challenge given its multiplicity of clinical presentations, variable combination of organ involvement and severity, and unpredictable longitudinal behaviour. Six manifestations of sarcoidosis are especially difficult to manage because of (i) an incomplete knowledge of causation - fatigue and small fibre neuropathy, (ii) the rare occurrence in sarcoidosis - intra-abdominal complications or (iii) the potentially life-threatening consequences in some patients - neurological disease, pulmonary hypertension and hypercalcaemia. In none of these situations have a prospective, double-blind, placebo-controlled trial of any therapy been conducted. Despite this absence of any firm evidence base to support therapeutic recommendations, these six entities can be extremely problematic for the practising clinician. It is for this reason that we have focused in this review on these six disease manifestations and provided a synopsis of each problem together with suggested treatment approaches, based on an analysis of the current literature.
由于其多种临床表现、器官受累和严重程度的不同组合以及不可预测的纵向行为,结节病可能是一个重大的治疗挑战。由于以下原因,结节病的六种表现尤其难以管理:(i) 对病因的不完全了解——疲劳和小纤维神经病,(ii) 在结节病中罕见发生——腹腔并发症,或 (iii) 在某些患者中潜在的危及生命的后果——神经系统疾病、肺动脉高压和高钙血症。在这些情况下,都没有针对任何治疗进行前瞻性、双盲、安慰剂对照试验。尽管缺乏任何支持治疗建议的确凿证据基础,但这些六种情况对于临床医生来说可能是极其棘手的问题。正是出于这个原因,我们在这篇综述中重点关注这六种疾病表现,并根据对当前文献的分析,对每个问题进行了概要介绍,并提出了治疗方法。