Parmar Manpreet K, Alikhan Mariam, Hsu Vivien M, Borham Amanda
Robert Wood Johnson Medical School, Piscataway Township, NJ, USA.
Case Rep Rheumatol. 2019 Jan 16;2019:7609386. doi: 10.1155/2019/7609386. eCollection 2019.
Granulomatosis with polyangiitis (GPA) is a rare ANCA-associated necrotizing granulomatous vasculitis affecting small- to medium-sized vessels. Common manifestations of this disease process affect the ear, nose, throat, upper and lower airways, and kidneys. Cardiac involvement has been reported in 6-44% of patients, primarily as coronary arteritis and pericarditis. A majority of case reports of pericardial effusions in patients with GPA identify patients having constrictive pericarditis secondary to uremia. We are presenting a case of hemorrhagic pericarditis in a patient with GPA in which the underlying inflammatory vasculitis likely played the primary role in the patient's presentation. Echocardiographic abnormalities have been found in 80% of patients with GPA. Given the high mortality from cardiac involvement in patients with GPA, screening echocardiograms for this patient population may serve as a helpful tool in gauging disease severity, thereby guiding therapy to prevent serious cardiac complications, such as cardiac tamponade as presented in this case report.
肉芽肿性多血管炎（GPA）是一种罕见的抗中性粒细胞胞浆抗体（ANCA）相关性坏死性肉芽肿性血管炎，累及中小血管。该疾病过程的常见表现累及耳、鼻、喉、上呼吸道和下呼吸道以及肾脏。据报道，6%至44%的患者有心脏受累，主要表现为冠状动脉炎和心包炎。大多数关于GPA患者心包积液的病例报告指出，患者继发于尿毒症的缩窄性心包炎。我们报告一例GPA患者发生出血性心包炎，其中潜在的炎症性血管炎可能在患者的临床表现中起主要作用。80%的GPA患者发现有超声心动图异常。鉴于GPA患者心脏受累的高死亡率，对该患者群体进行超声心动图筛查可能有助于评估疾病严重程度，从而指导治疗以预防严重的心脏并发症，如本病例报告中出现的心脏压塞。
