Kosugi Shumpei, Okada Masako, Iwata Keiji, Hasegawa Shinji
Department of Cardiology, Japan Community Healthcare Organization Osaka Hospital, 4-2-78 Fukushima, Fukushima-ku, Osaka, Japan.
Department of Clinical Laboratory, Japan Community Healthcare Organization Osaka Hospital, 4-2-78 Fukushima, Fukushima-ku, Osaka, Japan.
Eur Heart J Case Rep. 2018 Jul 31;2(3):yty087. doi: 10.1093/ehjcr/yty087. eCollection 2018 Sep.
Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease characterized by the tumefactive lesions and infiltration of IgG4-positive plasma cells. IgG4-RD has been described in various organs, but rarely the aortic valve. There are only a few reports of aortic stenosis, and none on significant aortic regurgitation. In addition, previous case reports relating to aortic valve lesions led to surgery as a first-line treatment. The effect of steroid treatment has not yet been determined.
A 62-year-old man, receiving steroid therapy, who presented with general malaise, shortness of breath, and bradycardia. He had suspected IgG4-RD because of pancreatitis, lacrimal gland enlargement, and retroperitoneal fibrosis. An examination revealed a thickened aortic valve extending to the left ventricular outflow tract with severe aortic regurgitation and complete atrioventricular block. He received intensive steroid therapy for a suspected IgG4-related aortic valve lesion. The complete atrioventricular block improved, but worsening aortic regurgitation caused congestive heart failure. He required replacement of the aortic valve. A histopathological examination of the excised aortic valve leaflets revealed IgG4-positive lymphoplasmacytic infiltration with fibrotic tissue. The prosthetic valve was functioning well without leakage around the valve at the 1-year follow-up.
This case highlights the rare possibility that IgG4-RD of the aortic valve also causes significant aortic regurgitation. Conservative treatment with steroids may induce regression of the lesion and contribute to the stability of the prosthetic valve after surgery, but it may also exacerbate heart failure due to the progression of aortic regurgitation in patients with aortic valve lesions.
免疫球蛋白G4相关性疾病（IgG4-RD）是一种以肿胀性病变和IgG4阳性浆细胞浸润为特征的全身性疾病。IgG4-RD已在多个器官中被描述，但累及主动脉瓣的情况罕见。仅有少数关于主动脉瓣狭窄的报道，而关于严重主动脉瓣反流的报道尚无。此外，先前有关主动脉瓣病变的病例报告将手术作为一线治疗方法。类固醇治疗的效果尚未确定。
一名62岁男性，正在接受类固醇治疗，出现全身不适、呼吸急促和心动过缓。由于胰腺炎、泪腺肿大和腹膜后纤维化，怀疑患有IgG4-RD。检查发现主动脉瓣增厚并延伸至左心室流出道，伴有严重主动脉瓣反流和完全性房室传导阻滞。他因疑似IgG4相关性主动脉瓣病变接受了强化类固醇治疗。完全性房室传导阻滞有所改善，但主动脉瓣反流恶化导致充血性心力衰竭。他需要更换主动脉瓣。对切除的主动脉瓣小叶进行组织病理学检查发现有IgG4阳性淋巴浆细胞浸润及纤维化组织。在1年随访时，人工瓣膜功能良好，瓣膜周围无渗漏。
该病例突出了一种罕见的可能性，即主动脉瓣的IgG4-RD也可导致严重主动脉瓣反流。类固醇保守治疗可能促使病变消退，并有助于术后人工瓣膜的稳定，但也可能因主动脉瓣病变患者主动脉瓣反流进展而加重心力衰竭。
