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目的:探讨以心律失常为首要和主要表现的结节病患者的临床特征。方法:回顾性分析北京协和医院呼吸内科门诊2017年1月至2020年12月接诊并规律随诊的4例累及心脏的结节病患者资料，其中男1例，女3例，年龄42~58岁，平均51岁；分析其临床表现、诊疗经过及转归。结果:4例患者均以心律失常为首发或主要临床表现就诊：1例为Ⅲ°房室传导阻滞、1例为Ⅱ°Ⅱ型房室传导阻滞、2例为频发室性早搏和短阵室性心动过速。3例在心律失常的诊断过程中，发现胸部CT异常而同期诊断了呼吸系统结节病；1例在心律失常5年后因颈部淋巴结肿大而发现胸部CT异常诊断呼吸系统结节病。4例患者均经支气管镜检查获取组织、病理证实为上皮样细胞肉芽肿性炎。2例患者接受心肌灌注延迟成像核磁共振检查，均有典型的心脏结节病的影像学表现，3例患者经正电子发射计算机断层显像证实心脏受累。4例患者均经口服糖皮质激素、免疫抑制剂以及抗心律失常药物治疗后病情控制，2例接受了心脏起搏器植入术。结论:不明原因的高度房室传导阻滞、室性心律失常的中老年患者需要考虑到心脏结节病的可能。建议常规行胸部CT筛查；而对于疑诊患者建议完善心脏增强核磁和（或）PET检查来明确诊断心脏结节病的诊断；口服糖皮质激素和免疫抑制剂有效。

Objective:To describe the clinical characteristics of sarcoidosis patients with arrhythmia as the primary or main manifestation.Methods:We conducted a retrospective analysis of arrhythmia-onset sarcoidosis cases between January 2017 and December 2020. Their clinical manifestations, radiological features, treatment and prognosis were reviewed and analyzed.Results:This study consisted of 3 females and 1 male, with a mean age of 51 years (range from 42 to 58 years old). Arrhythmia was the first or main clinical manifestation for all 4 cases, involving Ⅲ° atrioventricular block (AVB) ( n=1), Ⅱ° type Ⅱ AVB ( n=1), and frequent ventricular premature beats and short array ventricular tachycardia ( n=2). Three cases were diagnosed with respiratory sarcoidosis simultaneously during the diagnostic evaluation for arrhythmia. One case was diagnosed with sarcoidosis because of abnormal chest CT images due to cervical lymph node enlargement 5 years after arrhythmia. All 4 cases were confirmed as presenting epithelioid cell granulomatous inflammation by bronchoscopic biopsies. Late gadolinium enhancement with cardiac magnetic resonance (LGE-CMR) imaging was arranged for two cases. Both of them had typical imaging findings of cardiac sarcoidosis. Three cases were confirmed of cardiac involvement through positron emission computed tomography (PET)-CT. None of the enrolled four cases were arranged with endomyocardial biopsy. All four cases were improved with oral corticosteroids, immunosuppressants and anti-arrhythmic medications. Two cases underwent cardiac pacemaker implantation. Conclusions:The possibility of cardiac sarcoidosis should be considered in middle-aged and elderly patients with unexplained high-degree AVB or ventricular arrhythmia. Chest CT is recommended for routine screening for those cases. LGE-CMR and/or PET-CT is recommended for them to confirm the diagnosis of cardiac sarcoidosis. Corticosteroids and immunosuppressants are effective for these patients.