METHODS: We searched for malignancies in the SARCOGEAS cohort, a multicenter nationwide database of consecutive patients diagnosed with sarcoidosis according to the ATS/ESC/WASOG criteria. Solid malignancies were classified using the International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) nomenclature, and hematological malignancies using the 2016 WHO classification. We excluded patients with a biopsy-proven diagnosis of sarcoidosis based exclusively on demonstrating granulomas in tissues also involved by malignant cells.
RESULTS: Out of 1942 patients with sarcoidosis, 233 (12%) developed 250 malignancies, including solid (n = 173), hematological (n = 57), and both types of malignancies (n = 3). Concerning the time interval between the diagnoses of both conditions, 83 (36%) patients were diagnosed with malignancy at least 1 year before sarcoidosis diagnosis, 22 (9%) had s synchronous diagnosis of both diseases, and 118 (51%) developed malignancies at least 1 year after the diagnosis of sarcoidosis (the remaining cases developed malignancies in different time intervals). The multivariate-adjusted model showed that individuals with sarcoidosis who developed a malignancy had an hazard ratio (HR) of 2.27 [95% confidence interval (CI), 1.62-3.17] for having an asymptomatic clinical phenotype at diagnosis of sarcoidosis and that spleen (presence vs. absence: HR = 2.06; 95% CI, 1.21-3.51) and bone marrow (presence vs. absence: HR = 3.04; 95% CI, 1.77-5.24) involvements were independent predictors for the development of all-type malignancies. No predictive factors were identified when the analysis was restricted to the development of solid malignancies. The analysis limited to the development of hematological malignancies confirmed the presence of involvement in the spleen (HR = 3.73; 95% CI, 1.38-10.06) and bone marrow (presence vs. absence: HR = 8.00; 95% CI, 3.15-20.35) at the time of sarcoidosis diagnosis as predictive factors.
CONCLUSIONS: It is essential to consider the synchronous or metachronous timing of the diagnosis of malignancies in people with sarcoidosis. We found that half of the malignancies were diagnosed after a diagnosis of sarcoidosis, with spleen and bone marrow involvement associated with a four to eight times higher risk of developing hematological malignancies. Key messages What is already known on this topic Malignancies are one of the comorbidities more frequently encountered in people with sarcoidosis What this study adds Malignancies occur in 12% of patients with sarcoidosis Malignancy may precede, coincide with, or follow the diagnosis of sarcoidosis One-third were identified before sarcoidosis, and half were diagnosed after Spleen and bone marrow involvement are risk factors for developing hematological malignancies How this study might affect research, practice or policy Patients with sarcoidosis should be regularly monitored for neoplasms, informed of the increased risk, and educated on early detection. Those with spleen or bone marrow involvement must be closely followed.
方法:我们在SARCOGEAS队列中搜索恶性肿瘤,根据ATS/ESC/WASOG标准诊断为结节病的连续患者的多中心全国性数据库.使用国际疾病和相关健康问题统计分类第10修订版(ICD-10)命名法对实体恶性肿瘤进行分类,和使用2016年WHO分类的血液恶性肿瘤。我们排除了活检证实的结节病诊断的患者,仅基于在组织中表现出肉芽肿也与恶性细胞有关。
结果:在1942例结节病患者中,233(12%)发展了250个恶性肿瘤,包括固体(n=173),血液学(n=57),和两种类型的恶性肿瘤(n=3)。关于两种情况的诊断之间的时间间隔,83(36%)患者在结节病诊断前至少1年被诊断为恶性肿瘤,22人(9%)对两种疾病都有同步诊断,118例(51%)在诊断结节病后至少1年出现恶性肿瘤(其余病例在不同时间间隔出现恶性肿瘤).多变量调整模型显示,结节病患者发生恶性肿瘤的风险比(HR)为2.27[95%置信区间(CI),1.62-3.17]在诊断结节病和脾脏时具有无症状的临床表型(存在与缺席:HR=2.06;95%CI,1.21-3.51)和骨髓(存在vs.不存在:HR=3.04;95%CI,1.77-5.24)参与是所有类型恶性肿瘤发展的独立预测因子。当分析仅限于实体恶性肿瘤的发展时,没有发现预测因素。仅限于血液恶性肿瘤发展的分析证实了脾脏(HR=3.73;95%CI,1.38-10.06)和骨髓(存在与不存在:HR=8.00;95%CI,3.15-20.35)在结节病诊断时作为预测因素。
结论:必须考虑结节病患者诊断恶性肿瘤的同步或异时时机。我们发现一半的恶性肿瘤是在诊断结节病后被诊断出来的,脾脏和骨髓受累与发展为血液系统恶性肿瘤的风险高4至8倍。关键信息关于这个主题已经知道的恶性肿瘤是结节病患者中更常见的合并症之一这项研究增加了恶性肿瘤发生在12%的结节病患者中恶性肿瘤可能先于,与,或跟随结节病的诊断三分之一是在结节病之前确定的,和一半被诊断后,脾脏和骨髓受累是发展血液系统恶性肿瘤的危险因素。实践或政策结节病患者应定期监测肿瘤,得知风险增加,并接受了早期检测的教育。必须密切关注脾脏或骨髓受累的患者。