Menz V, Drude L, Schönian U, Herzum M, Bethge C, Maisch B
Abteilung Innere Medizin-Kardiologie, Philipps-Universität Marburg.
Herz. 1994 Jun;19(3):138-43.
The clinical and immunological 8-year follow-up of a 45-year old female with hypereosinophilic syndrome and cardiac involvement of Löffler's fibroplastic endocarditis which was complicated by infective endocarditis during immunosuppressive treatment is presented. All 3 stages of the disease were documented by clinical and histologic data: stage 1 by biopsy proven eosinophilic myocarditis and arteritis, stage 2 with Berlin blue positive parietal thrombosis, and the fibrotic features of stage 3 both by fibrosis in the latest biopsies and by echocardiography. It was remarkable, however, that this classic clinical case of Löffler's endocarditis lacked a few serological markers postulated to be found regularly, e.g., a positive staining for the cationic protein and major ribonucleases. Instead, the patient demonstrated all the immunological features of autoreactive myocarditis with cytolytic, complement fixing antimyolemmal antibodies. After an initial loading dose of 120 mg prednisolone per day for 6 weeks (11/1986), the steroid dosage was reduced to 40 mg (12/1986) and diminished to 15 mg/day in 1988 to 1989. Finally, the patient was on 4 mg prednisolone per day for almost 4 years. In 1987, azathioprine was added in the dose of 150 mg/day for 6 weeks. In 3/1994 the patient developed infective endocarditis with streptococcus sanguis and presented with dyspnoea. By echocardiography a large floating structure was diagnosed on the anterior mitral leaflet and the left atrium was enlarged by severe mitral regurgitation. Infective endocarditis was successfully treated with antibiotics, but mitral regurgitation made valve replacement obligatory.
本文介绍了一名45岁女性高嗜酸性粒细胞综合征患者的临床和免疫学8年随访情况,该患者患有Löffler纤维增生性心内膜炎并累及心脏,在免疫抑制治疗期间并发感染性心内膜炎。疾病的所有三个阶段均由临床和组织学数据记录:第1阶段通过活检证实为嗜酸性粒细胞性心肌炎和动脉炎,第2阶段有柏林蓝阳性的壁层血栓形成,第3阶段的纤维化特征通过最新活检中的纤维化以及超声心动图得以体现。然而,值得注意的是,这个经典的Löffler心内膜炎临床病例缺乏一些假定会经常出现的血清学标志物,例如阳离子蛋白和主要核糖核酸酶的阳性染色。相反,该患者表现出自反应性心肌炎的所有免疫学特征,伴有溶细胞、补体固定的抗肌膜抗体。在最初每天给予120毫克泼尼松龙负荷剂量6周(1986年11月)后,类固醇剂量减至40毫克(1986年12月),并在1988年至1989年减至每天15毫克。最后,患者每天服用4毫克泼尼松龙近4年。1987年,添加硫唑嘌呤,剂量为每天150毫克,持续6周。1994年3月,患者发生血链球菌感染性心内膜炎并出现呼吸困难。通过超声心动图在二尖瓣前叶诊断出一个大的漂浮结构,由于严重二尖瓣反流左心房扩大。感染性心内膜炎通过抗生素成功治疗,但二尖瓣反流使得瓣膜置换成为必要。