结节病

0 期：正常

I 期：双侧肺门淋巴结肿大[Figure caption and citation for the preceding image starts]: 双侧肺门淋巴结肿大（图片）来自 M.P.Muthiah；经许可后使用 [Citation ends].

II 期：双侧肺门淋巴结肿大加肺浸润

III 期：肺浸润，无肺门淋巴结肿大

IV 期：广泛纤维化和结构破坏变形。

可见肺门和/或气管旁淋巴结肿大，上叶为主、双侧浸润；可见胸膜腔积液（罕见）和蛋壳钙化（非常罕见）

通常继发于脾和/或骨髓受累。[1]American Thoracic Society. Statement on sarcoidosis: joint statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG), adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Crit Care Med. 1999;160:736-755.http://www.atsjournals.org/doi/full/10.1164/ajrccm.160.2.ats4-99http://www.ncbi.nlm.nih.gov/pubmed/10430755?tool=bestpractice.com

4%-20% 的患者出现贫血；40% 的患者出现白细胞减少

如果发生肾脏受累。

可能会升高

如果发生肾脏受累。

可能会升高

可能出现无症状性转氨酶 [天门冬氨酸氨基转移酶 (AST) 和丙氨酸氨基转移酶 (ALT) ] 水平升高。

增高

由激活的巨噬细胞和肉芽肿造成的骨化三醇生成失调所导致。

高钙血症

用来监测疾病。

用力肺活量持续下降说明疾病进展。[1]American Thoracic Society. Statement on sarcoidosis: joint statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG), adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Crit Care Med. 1999;160:736-755.http://www.atsjournals.org/doi/full/10.1164/ajrccm.160.2.ats4-99http://www.ncbi.nlm.nih.gov/pubmed/10430755?tool=bestpractice.com

限制性或阻塞性或混合性通气障碍

用于排除或确认心脏受累。

近半数心脏结节病患者都出现心律、传导或复极异常。[15]Hirose Y, Ishida Y, Hayashida K, et al. Myocardial involvement in patients with sarcoidosis. An analysis of 75 patients. Clin Nucl Med. 1994;19:522-526.http://www.ncbi.nlm.nih.gov/pubmed/8062473?tool=bestpractice.com

传导障碍

结节病完全无反应或仅有部分反应，继发于细胞免疫功能抑制。[16]Bianco A, Spiteri MA. Peripheral anergy and local immune hyperactivation in sarcoidosis: a paradox or birds of a feather. Clin Exp Immunol. 1997;110:1-3.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1904788/pdf/cei0110-0001.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/9353140?tool=bestpractice.com

阴性

磨玻璃样外观可能说明患有潜在可逆性疾病。

囊性结构破坏表明患有不可逆转的疾病。[17]Murdoch J, Muller NL. Pulmonary sarcoidosis: changes on follow-up CT examination. AJR Am J Roentgenol. 1992;159:473-477.http://www.ajronline.org/doi/pdf/10.2214/ajr.159.3.1503008http://www.ncbi.nlm.nih.gov/pubmed/1503008?tool=bestpractice.com

在支气管血管分布里出现肺门和/或气管旁淋巴结肿大，上叶为主、双侧浸润；长期疾病患者会出现肺门钙化或纵隔淋巴结

在大多数情况下，对于诊断必不可少。

从大的纵隔淋巴结处（如果存在）进行经支气管针吸活检。[18]Bradley B, Branley HM, Egan JJ, et al. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax. 2008;63(suppl 5):v1-v58.http://www.ncbi.nlm.nih.gov/pubmed/18757459?tool=bestpractice.com[Figure caption and citation for the preceding image starts]: 显微照片显示典型结节病结构完整的肉芽肿（图片）来自 M.P.Muthiah；经许可后使用 [Citation ends].

非干酪样肉芽肿

特异性为 94%。敏感性为 53%。[1]American Thoracic Society. Statement on sarcoidosis: joint statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG), adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Crit Care Med. 1999;160:736-755.http://www.atsjournals.org/doi/full/10.1164/ajrccm.160.2.ats4-99http://www.ncbi.nlm.nih.gov/pubmed/10430755?tool=bestpractice.com

BAL 淋巴细胞增多，CD4/CD8 比值大于 3.5

对可疑结节病的病损进行皮肤活检，可从组织学上确诊。

非干酪样肉芽肿

肉芽肿内的巨噬细胞导致肉芽肿和维生素 D 调节异常，造成高钙尿症。[19]Sharma OP. Vitamin D, calcium, and sarcoidosis. Chest. 1996;109:535-539.http://www.ncbi.nlm.nih.gov/pubmed/8620732?tool=bestpractice.com

高钙尿症

近半数患者水平增高。

不一定反映疾病活动度。

其实用价值不可靠且有争议。[1]American Thoracic Society. Statement on sarcoidosis: joint statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG), adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Crit Care Med. 1999;160:736-755.http://www.atsjournals.org/doi/full/10.1164/ajrccm.160.2.ats4-99http://www.ncbi.nlm.nih.gov/pubmed/10430755?tool=bestpractice.com

升高

典型的模式包括熊猫征：泪腺和腮腺摄取；λ 征：肺门旁、肺下叶和右气管旁或奇静脉淋巴结摄取。[10]Nunes H, Brillet PY, Valeyre D, et al. Imaging in sarcoidosis. Semin Respir Crit Care Med. 2007;28:102-120.http://www.ncbi.nlm.nih.gov/pubmed/17330195?tool=bestpractice.com

缺乏特异性。有时需要进行 48 小时序列成像。

该试验的临床价值仍有争议。

可能显示典型的摄取模式

气管内超声引导下针吸活检术是诊断纵隔淋巴结肿大的诊疗标准和常规实践，具有高敏感性 (88%) 和高特异性 (100%)。[7]Varela-Lema L, Fernández-Villar A, Ruano-Ravina A. Effectiveness and safety of endobronchial ultrasound-transbronchial needle aspiration: a systematic review. Eur Respir J. 2009;33:1156-1164.http://erj.ersjournals.com/content/33/5/1156.longhttp://www.ncbi.nlm.nih.gov/pubmed/19407050?tool=bestpractice.com[8]National Institute for Health and Care Excellence. Endobronchial ultrasound-guided transbronchial needle aspiration for mediastinal masses. February 2008. http://www.nice.org.uk/ (last accessed 30 August 2017).https://www.nice.org.uk/guidance/ipg254[9]Agarwal R, Srinivasan A, Aggarwal AN, et al. Efficacy and safety of convex probe EBUS-TBNA in sarcoidosis: a systematic review and meta-analysis. Respir Med. 2012;106:883-892.http://www.ncbi.nlm.nih.gov/pubmed/22417738?tool=bestpractice.com[20]Agarwal R, Aggarwal AN, Gupta D. Efficacy and safety of conventional transbronchial needle aspiration in sarcoidosis: a systematic review and meta-analysis. Respir Care. 2013;58:683-693.http://rc.rcjournal.com/content/58/4/683.fullhttp://www.ncbi.nlm.nih.gov/pubmed/23050747?tool=bestpractice.com[21]von Bartheld MB, Dekkers OM, Szlubowski A, et al. Endosonography vs conventional bronchoscopy for the diagnosis of sarcoidosis: the GRANULOMA randomized clinical trial. JAMA. 2013;309:2457-2464.http://jama.jamanetwork.com/article.aspx?articleid=1697962http://www.ncbi.nlm.nih.gov/pubmed/23780458?tool=bestpractice.com

非干酪样肉芽肿

可能有助于识别结节病受累部位，包括心脏和大脑。[10]Nunes H, Brillet PY, Valeyre D, et al. Imaging in sarcoidosis. Semin Respir Crit Care Med. 2007;28:102-120.http://www.ncbi.nlm.nih.gov/pubmed/17330195?tool=bestpractice.com[Figure caption and citation for the preceding image starts]: A) 心脏（短轴）钆增强 MRI 扫描显示前间壁心肌出现延迟增强源自 http://casereports.bmj.com/cgi/content/full/2009/feb16_1/bcr2006070805 [Citation ends].

心脏发现包括结节性病变和/或局灶性心肌增厚；大脑发现呈多样化和非特异性，包括脑膜增厚（伴或不伴脑室周围白质病变）

现已证明 PET 扫描可发现结节病的特征性摄取模式，并有助于心脏结节病的诊断。[11]Youssef G, Leung E, Mylonas I, et al. The use of 18F-FDG PET in the diagnosis of cardiac sarcoidosis: a systematic review and metaanalysis including the Ontario experience. J Nucl Med. 2012;53:241-248.http://www.ncbi.nlm.nih.gov/pubmed/22228794?tool=bestpractice.com其创伤性比心内膜心肌活检小得多，操作相关的并发症极少或没有。[12]Nobuhiro T, Atsuko T, Yoshikazu N, et al. Heterogeneous myocardial FDG uptake and the disease activity in cardiac sarcoidosis. JACC Cardiovasc Imaging. 2010;3:1219-1228.http://www.ncbi.nlm.nih.gov/pubmed/21163450?tool=bestpractice.com[Figure caption and citation for the preceding image starts]: (B) 心脏（短轴）18F-FDG PET 扫描显示，与肉芽肿性炎症相符的前间壁内局灶摄取。(C) 从心底到心尖（从左向右）的心脏（短轴）18F-FDG PET 扫描显示，在基线状态前间壁内的局灶摄取（上部系列）和治疗后摄取消失（下部系列）源自 http://casereports.bmj.com/cgi/content/full/2009/feb16_1/bcr2006070805 [Citation ends].

当存在诊断/监测方面的不确定性时，也可用于评估基线状态的疾病活动度和分布以及评估疾病反应。[22]Royal College of Radiologists; Royal College of Physicians of London. Evidence-based indications for the use of PET-CT in the United Kingdom 2016. 2016. http://www.rcr.ac.uk (last accessed 30 August 2017).https://www.rcr.ac.uk/publication/evidence-based-indications-use-pet-ct-united-kingdom-2016

异质心肌 FDG 摄取