Carbajal Hector, Waters Lindsay, Popovich Jovan, Boniuk Milton, Chevez-Barrios Patricia, Marcus Donald M, Sessoms Sandra
Houston Methodist Hospital, Houston, Texas ; Weill Cornell Medical College, New York, New York.
Methodist Debakey Cardiovasc J. 2013 Oct-Dec;9(4):230-2. doi: 10.14797/mdcj-9-4-230.
IgG4-related systemic disease is an inflammatory disorder that can affect many organs. This case report describes a patient who in 2004 was found to have an inflammatory pseudotumor with IgG4 pathology. Over the next 3 years, visual symptoms responded well to recurrent courses of prednisone. In 2009, the patient developed chest pain and bradycardia with subsequent third-degree heart block, necessitating placement of a pacemaker. A subsequent PET scan showed extensive involvement of multiple organs as described in IgG4 disease as well as involvement of the myocardium and SA node. Pseudotumors involving the heart have been reported but have not been shown to be related to IgG4 disease. Although there was no pathology confirmation of heart involvement, the nature and extent of the organ involvement led us to conclude that it was due to IgG4-related disease. The use of the PET scan may help identify involvement of the myocardium.
IgG4相关性系统性疾病是一种可累及多个器官的炎症性疾病。本病例报告描述了一名患者，该患者在2004年被发现患有具有IgG4病理特征的炎性假瘤。在接下来的3年里，视觉症状对反复使用泼尼松治疗反应良好。2009年，该患者出现胸痛和心动过缓，随后发展为三度心脏传导阻滞，需要植入起搏器。随后的PET扫描显示多个器官广泛受累，符合IgG4疾病的表现，同时心肌和窦房结也受累。虽然有报道称存在累及心脏的假瘤，但尚未证明其与IgG4疾病有关。尽管没有心脏受累的病理证实，但器官受累的性质和程度使我们得出结论，这是由IgG4相关性疾病引起的。PET扫描的应用可能有助于识别心肌受累情况。
