Diaz Charyse, Lim Matthew A, Liu Chloe A, Miwa Chloe S, Tokunaga Darcy, Hamamura Faith D, Yamamoto Kara, Kurahara David
Department of Pediatrics, University of Hawaii, John A. Burns School of Medicine, Kapi'olani Medical Center for Women and Children, Honolulu, HI 96826, USA.
Case Rep Pediatr. 2018 Dec 30;2018:8762482. doi: 10.1155/2018/8762482. eCollection 2018.
The presence of rheumatic heart disease (RHD) and systemic lupus erythematosus (SLE) has rarely been described in one patient. This report describes an adolescent Polynesian male with RHD who developed SLE years later. Initially, he fulfilled modified Jones criteria for rheumatic fever with aortic insufficiency, transient arthritis, elevated streptococcal titers, and a high erythrocyte sedimentation rate with a negative antinuclear antibody (ANA). He responded well to nonsteroidal anti-inflammatory and penicillin prophylaxis, which supported the diagnosis of rheumatic fever. Five years after his RHD diagnosis, he developed pancreatitis with glomerulonephritis, nephrosis, and pancytopenia. In addition, laboratory results revealed that he had multiple autoantibodies: anti-Sm and extremely elevated anti-dsDNA and ANA, fulfilling diagnostic criteria for SLE. The patient was treated, and he responded to pulse steroids followed by oral steroid therapy. To our knowledge, there are no known reported cases of a patient who was diagnosed with both RHD and SLE and met the clinical criteria for both diseases. The rarity of this concurrent disease process in one patient suggests a possible overlap in humoral immunity toward self-antigens as well as ethnic variability that increases predisposition to rheumatologic diseases.
风湿性心脏病(RHD)和系统性红斑狼疮(SLE)同时出现在一名患者身上的情况鲜有报道。本报告描述了一名患有RHD的波利尼西亚青少年男性,数年后患上了SLE。最初,他符合风湿热的改良琼斯标准,伴有主动脉瓣关闭不全、短暂性关节炎、链球菌滴度升高、红细胞沉降率升高且抗核抗体(ANA)阴性。他对非甾体抗炎药和青霉素预防治疗反应良好,这支持了风湿热的诊断。在被诊断为RHD五年后,他出现了胰腺炎,并伴有肾小球肾炎、肾病和全血细胞减少。此外,实验室检查结果显示他有多种自身抗体:抗Sm抗体以及抗双链DNA和ANA极度升高,符合SLE的诊断标准。该患者接受了治疗,对脉冲类固醇治疗有反应,随后接受口服类固醇治疗。据我们所知,尚无已知的报告病例显示患者同时被诊断为RHD和SLE且符合两种疾病的临床标准。这种并发疾病过程在一名患者中的罕见性表明,针对自身抗原的体液免疫可能存在重叠,以及种族差异增加了患风湿性疾病的易感性。