Immunologic and immunogenetic studies in rheumatic fever and rheumatic heart disease

In order to evaluate all the important limbs of the immune system in the same patient population with rheumatic fever (RF) and rheumatic heart disease (RHD) cellular and humoral immune parameters as well as the immunogenetic profile in 265 North Indian patients with RHD were evaluated. They were studied for class in HLA antigens and 165 of them were also evaluated for the class II (DR locus) antigen profile. Data obtained was compared with 400 and 134 healthy controls respectively of the same ethnicity. Humoral immune parameters (Serum immunoglobulins IgG, IgA; Serum complement fractions C3, C4, C3d; circulating immune complexes and B lymphocyte numbers) and cellular immune parameters (total leucocyte and lymphocyte counts; T lymphocyte sub-populations-CD4, CD8 counts; lymphocyte migration inhibition to an extracellular streptococcal antigen, streptolysin ’O’) were studied in 23 patients with RF, 21 patients with “inactive” RHD and 20 normal controls. Patients of RHD were noted to have an increased frequency of DR3 (P < 0.001; Relative risk = 2.3) and a decreased frequency of DR2 (P < 0.001; Relative risk = 0.3) as compared to the controls. Patients of RF had evidence of an altered regulatory T cell function (Increased CD4/CD8 ratio) and decreased cell mediated immunity to streptolysin ‘0’. An increased humoral immune response (increased B cell counts, elevated serum IgG, circulating immune complexes and C3d) was noted in patients of RF as well as “inactive” RHD. An integrated pathogenetic model with immune response associated antigens of the DR locus influencing selection of cardiac cross-reactive antigens by the antigen processing macrophages, an altered regulatory T cell function with decreased suppressor T cell activity leading to an abnormal immune response is proposed to explain the pathogenesis of RF.     

链球菌感染后风湿热和风湿性心脏病

风湿热(rheumatic fever)是儿童上呼吸道或皮肤等感染A族链球菌(group A streptococcus,GAS)后引起的一种自身免疫性疾病。在结缔组织有多发的非化脓性病变,主要累及心脏和关节,脑、皮肤、粘膜、血管、肺、肾等亦可受累,而心脏为本病唯一留有后遗症的器官,可导致永久性瓣膜损害、心力衰竭,甚至死亡。一、流行病学风湿热初次发病以儿童和青少年为多见,4岁前及25～30岁以后发病者少见,女性发病似较男性多见。在发达国家风湿热和风湿性心脏病的发病率已大幅下降,在发展中国家仍居高不下。近几十年来,国外报告GAS感染激增,我国也有明显的上…     

Mortality and hospitalisation costs of rheumatic fever and rheumatic heart disease in New Zealand

Aims: To estimate the annual mortality and the cost of hospital admissions for acute rheumatic fever (ARF) and rheumatic heart disease (RHD) for New Zealand residents. Methods: Hospital admissions in 2000–2009 with a principal diagnosis of ARF or RHD (ICD9_AM 390‐398; ICD10‐AM I00‐I099) and deaths in 2000–2007 with RHD as the underlying cause were obtained from routine statistics. The cost of each admission was estimated by multiplying its diagnosis‐related group (DRG) cost weight by the national price for financial year 2009/2010. Results: There were on average 159 RHD deaths each year with a mean annual mortality rate of 4.4 per 100 000 (95% confidence limit 4.2, 4.7). Age‐adjusted mortality was five‐ to 10‐fold higher for Māori and Pacific peoples than for non‐Māori/Pacific. The mean age at RHD death (male/female) was 56.4/58.4 for Māori, 50.9/59.8 for Pacific and 78.2/80.6 for non‐Māori, non‐Pacific men and women. The average annual DRG‐based cost of hospital admissions in 2000–2009 for ARF and RHD across all age groups was $12.0 million (95% confidence limit $11.1 million, $12.8 million). Heart valve surgery accounted for 28% of admissions and 71% of the cost. For children 5–14 years of age, valve surgery accounted for 7% of admissions and 27% of the cost. Two‐thirds of the cost occurs after the age of 30. Conclusions: ARF and RHD comprise a burden of mortality and hospital cost concentrated largely in middle age. Māori and Pacific RHD mortality rates are substantially higher than those of non‐Māori/Pacific.     

儿童风湿热及风湿性心瓣膜病85例临床分析

为观察儿童风湿热及风湿性心瓣膜病临床动态变化趋势,分析本院90年代诊治的85例风湿热及心瓣膜病发生状况、临床特点和治疗预防复发情况。结果示本组风湿热造成急性心瓣膜炎占77.65%,首诊慢性心瓣膜病占22.35%,其中三分之二患儿以往无急性风湿热病史。首次发病复发率为14.12%,风湿活动复发常以不同程度心力衰竭（心衰）症状就诊,激素抗风湿治疗能够迅速缓解急性心脏炎和改善心功能。1例反复风湿活动顽固性心力衰竭接受二尖瓣置换术效果甚佳。结果表明,减少儿童风湿性心瓣膜病形成不仅要控制急性风湿热,而且需要及时发现潜隐的风湿活动。     

357例风湿热和风湿性心脏瓣膜病30年临床变迁

目的 探讨30年来风湿热和风湿性心脏瓣膜病(风心病)的发病特点及规律，为临床提供诊断和治疗依据。方法 对我科1972～2002年357例风湿热及风心病住院患儿的流行病学、临床表现、实验室检查、并发症及预后进行回顾性分析。结果 儿童风湿热和风心病住院人数有增加趋势，但其在流行病学方面改变不大。结论 30年来风湿热的发病及严重程度未下降，1992年修订的Jones标准有待进一步完善。     

Rheumatic fever and rheumatic heart disease in children in North India

Summary Data on 118 cases of rheumatic fever and rheumatic heart disease in the age group of 4–14 years are reported. 52.8% gave a past history of rheumatic fever or rheumatic heart disease. 72% cases showed evidence of rheumatie activity. Carditis was the commonest lesion seen in 71% patients. 56.8% cases were in congestive heart failure. Chorea and subcutaneous nodules were seen in 17.8% and 16.1% respectively—far more frequent than generally believed in our country. The presence of subcutaneous nodules without cardiac involvement and of arthritis or arthralgia in association with chorea seen in some children are reported. From the Department of Paediatrics and Cardiology, Post-graduate Institute of Medical Education and Research, Chandigarh-11.