Department of Medicine, University of Cincinnati Medical Center, Cincinnati, Ohio, USA.
Royal Hospital Brompton, London, UK.
Curr Opin Pulm Med. 2019 Sep;25(5):497-504. doi: 10.1097/MCP.0000000000000612.
Advanced sarcoidosis is an important cause of morbidity and mortality in sarcoidosis. Over the past few years, several studies have been published clarifying the prevalence and severity of this condition.
Pulmonary involvement is the most common form of sarcoidosis. Increased morbidity and significant mortality is encountered in advanced lung disease. Although many sarcoidosis patients with pulmonary fibrosis have a normal life expectancy, at least 20% develop progression and may die from this complication. Sarcoidosis-associated pulmonary hypertension (SAPH) is an independent cause of death in advanced pulmonary sarcoidosis. Two large multicenter registries and a large single-center report provide more details regarding presentation and outcome of SAPH. Advanced neurologic disease is associated with significant morbidity, but not much mortality. Two large retrospective reviews demonstrated the effectiveness of infliximab in treating advanced neurosarcoidosis. Advanced cardiac sarcoidosis can lead to mortality.
Advanced sarcoidosis is associated with significant morbidity and some mortality. Up to a quarter of all sarcoidosis patients have one or more forms of advanced disease. These patients require closer monitoring and often multiples treatments.
晚期结节病是结节病发病率和死亡率的重要原因。在过去的几年中，已经发表了几项研究阐明了这种情况的患病率和严重程度。
肺部受累是结节病最常见的形式。晚期肺病会导致发病率增加和显著的死亡率。尽管许多患有肺纤维化的结节病患者具有正常的预期寿命，但至少有 20%的患者会出现进展，并可能因此并发症而死亡。结节病相关性肺动脉高压（SAPH）是晚期肺结节病死亡的独立原因。两项大型多中心登记研究和一项大型单中心报告提供了有关 SAPH 表现和结局的更多细节。晚期神经疾病与严重的发病率相关，但死亡率不高。两项大型回顾性研究表明英夫利昔单抗治疗晚期神经结节病的有效性。晚期心脏结节病可导致死亡。
晚期结节病与严重的发病率和一定的死亡率相关。多达四分之一的结节病患者存在一种或多种形式的晚期疾病。这些患者需要更密切的监测和经常接受多种治疗。
