Meyer A, Schäfer H, Döring V, Maisch B, Kirsten D
Medizinische Klinik, Universitäts-Krankenhauses Eppendorf, Hamburg.
Dtsch Med Wochenschr. 1999 Oct 1;124(39):1131-4. doi: 10.1055/s-2007-1024502.
A 40-year-old man with histologically proven sarcoidosis, known for 15 years, which had involved the myocardium was hospitalized because of intractable heart failure (NYHA class IV). An implantation of an intracardiac defibrillator for ventricular arrhythmias (Lown type IVa) had preceded. On physical examination severe dyspnea at rest cough and fever were noted.
The erythrocyte sedimentation rate (88/104 mm), C-reactive protein (250 mg/l) and white cell count (20/nl) were markedly increased. Serum sodium (113 mmol/l), potassium (3.0 mmol/l) and chloride (64 mmol/l) were markedly reduced, while creatinine (2.5 mg/dl) and urea (82 mg/dl) were elevated due to renal failure. The chest radiogram demonstrated central venous congestion, cardiomegaly and right pericardial infiltration. There were no obvious changes due to sarcoidosis and computed tomography did not indicate pulmonary involvement by sarcoidosis. The echocardiogram revealed severe impairment of left ventricular function with an ejection fraction of ca. 14%.
DIAGNOSIS, TREATMENT AND COURSE: Heart failure (NYHA class IV), caused by a dilated cardiomyopathy of sarcoidosis, was accompanied by pneumonia which responded to antibiotics. But the chronic heart failure failed to improve on drug treatment and cardiac transplantation was undertaken. The explanted myocardium was examined histologically, immunologically and virologically. Antibodies were demonstrated against vascular endothelium, sarcolemma and endocardium (IgG and IgA), but not by PCR against cytomegalovirus, enterovirus and adenovirus. The transplantation was without complication and, under azathioprine and methylprednisolone, one rejection had occurred until now. The patient has been working full-time since 2 years in his previous occupation of lorry driver. There has been no evidence of renewed sarcoidosis activity.
Sarcoidosis may involve the myocardium in up to 25% of cases. Clinically relevant symptoms are even more rare. Sarcoidosis should be included in the differential diagnosis of unexplained serious arrhythmias or cardiomyopathy, particularly in young persons. Cardiac transplantation may have to be contemplated if drug or pacemaker treatment fails to control heart failure.
一名40岁男性，组织学确诊结节病15年，累及心肌，因难治性心力衰竭（纽约心脏协会心功能IV级）入院。此前已植入心脏内除颤器以治疗室性心律失常（Lown IVa型）。体格检查发现患者静息时严重呼吸困难、咳嗽及发热。
红细胞沉降率（88/104mm）、C反应蛋白（250mg/l）及白细胞计数（20/nl）显著升高。血清钠（113mmol/l）、钾（3.0mmol/l）及氯（64mmol/l）显著降低，因肾衰竭肌酐（2.5mg/dl）及尿素（82mg/dl）升高。胸部X线片显示中心静脉淤血、心脏扩大及右心包浸润。结节病无明显变化，计算机断层扫描未显示肺部有结节病累及。超声心动图显示左心室功能严重受损，射血分数约为14%。
诊断、治疗与病程：结节病所致扩张型心肌病引起的心力衰竭（纽约心脏协会心功能IV级），伴有对抗生素有反应的肺炎。但慢性心力衰竭药物治疗无效，遂进行心脏移植。对切除的心肌进行了组织学、免疫学及病毒学检查。检测到针对血管内皮、肌膜及心内膜的抗体（IgG和IgA），但针对巨细胞病毒、肠道病毒及腺病毒的聚合酶链反应未检测到。移植无并发症，在硫唑嘌呤和甲泼尼龙治疗下，至今发生过一次排斥反应。患者自两年前起一直全职从事此前的卡车司机工作。无结节病复发活动的证据。
结节病累及心肌的病例可达25%。临床相关症状更为罕见。结节病应列入不明原因严重心律失常或心肌病的鉴别诊断，尤其是在年轻人中。如果药物或起搏器治疗无法控制心力衰竭，可能需要考虑心脏移植。
