缩窄性心包炎(CP)表现为一种病理生理状态,其中心包由于纤维化变化而变得无弹性。最常见的是继发于持续的炎症过程。该疾病的特征在于由于心包顺应性的丧失而导致的舒张心功能受损。免疫球蛋白G4(IgG4)相关疾病,以IgG4阳性浆细胞的隐伏增殖和随后的各种器官内的纤维化为标志的实体,是CP的罕见但公认的原因。一名55岁的男性患者的下肢呼吸困难和水肿的临床表现阐明了CP固有的诊断复杂性。超声心动图显示一系列迹象,包括环回复,间隔弹跳,和充血下腔静脉;心脏磁共振成像(MRI)显示弥漫性心包增厚伴钆增强延迟,提示长期炎症状态;右心导管检查证实了心脏腔舒张压CP均衡的血流动力学标志。血清学分析显示血清IgG4和IgE水平升高,指出IgG4相关疾病的鉴别诊断。鉴于IgG4相关CP的非特异性临床表现,增强的怀疑指数与系统的影像学和血清学评估方法相结合是最重要的。
Constrictive pericarditis (CP) presents as a pathophysiological state where the pericardium becomes inelastic due to fibrotic changes, most commonly secondary to a protracted inflammatory process. The disease is characterized by compromised diastolic cardiac function due to loss of pericardial compliance. Immunoglobulin G4 (IgG4)-related disease, an entity marked by the insidious proliferation of IgG4-positive plasma cells and subsequent fibrosis within various organs, is an infrequent but recognized cause of CP. A case of a 55-year-old male patient with clinical manifestations of dyspnea and edema in the lower extremities elucidates the diagnostic complexity inherent to CP. Echocardiography revealed a constellation of signs, including annulus reversus, septal bounce, and a congested inferior vena cava; cardiac magnetic resonance imaging (MRI) demonstrated diffuse pericardial thickening with delayed gadolinium enhancement, suggestive of a long-term inflammatory state; and right heart catheterization confirmed the hemodynamic hallmark of CP-equalization of diastolic pressures across the cardiac chambers. The serological analysis elicited elevated serum levels of IgG4 and IgE, pointing to the differential diagnosis of IgG4-related disease. Given the nonspecific clinical presentation of IgG4-related CP, a heightened index of suspicion combined with a systematic approach to imaging and serological evaluation is paramount.