DISCUSSION
Our case provides evidence of the persistence of acute rheumatic fever in Bhutan where clinical data and publication is very limited. In contrast to carditis and arthritis, which typically present within 21 days, the onset of Sydenham chorea usually occurs one to eight months after the inciting infection [2]. By the time patient present to medical care, they might not remember the inciting infection and therefore we cannot solely depend on history to make a diagnosis. Alternative aetiologies of acquired chorea such as autoimmune or inflammatory, cerebrovascular, drugs, infections, metabolic disorders or neoplasia should be kept in mind [2].
The diagnosis of rheumatic fever is based on the Jones criteria [Table 1]. The most common manifestation which is present in 80% of patients is arthritis, described as painful, migratory and transient. Frequently affected joints are knees and ankles. Carditis occurs in 40 - 75%, erythema marginatum and subcutaneous nodules are rare, occurring in less than 10% of patients [4]. Sydenham chorea is also a rare presentation, occurring in 10-30% [4]. In our case, the patient presented to us with Sydenham chorea which hints at a possible larger number of cases with acute rheumatic fever that may be underdiagnosed or under reported.
Carditis can be diagnosed clinically with the presence of an audible murmur consistent with aortic or mitral regurgitation on auscultation [2]. However, more recent studies on patients with acute RF have brought out the shortcomings of auscultation in identifying valve disease which does not result in hemodynamic abnormalities consisting of murmurs [4]. This has resulted in the identification of sub-clinical carditis by echocardiography [2]. In Bhutan, we have facility of echocardiography in only in three tertiary hospitals out of twenty districts in the country leading to high chances of missing the diagnosis of sub-clinical carditis.
In our case, a patient had sub-clinical carditis in the form of mitral regurgitation diagnosed with echocardiography. The patient had two major criteria, Sydenham chorea and sub-clinical carditis, and minor criteria, raised ESR and ASO titre thereby fulfilling the Jones criteria for rheumatic fever.
Rheumatic heart disease is preventable. It is a serious public health problem especially in low and middle income countries with limited capacities for the diagnosis and timely management of streptococcal infection [5]. It exerts massive economic effects globally, mainly because of premature death in children and working-age adults [7]. Globally, ARF and RHD are seen in developing nations or among disadvantaged populations within developed nations [8]. The global cost of deaths due to rheumatic heart disease in 2010 was estimated to be US$ 2200 billion (discounted) or US$ 5400 billion (undiscounted) [5]. The most devastating effects are on children and young adults in their most productive years because it leads to increased school absenteeism and drop-out, and lost wages [5]. The patient, in our case, she dropped out of school, but will be continuing in the next academic session after the control of the chorea.
The prevention, control and elimination of rheumatic heart disease is increasingly being recognized as an important developmental issue by the World Health Organization [5]. The barriers to prevention, control and elimination of rheumatic heart disease are poor primary and secondary prevention and access to primary health care, inadequate numbers and training of health workers at all levels, the neglect of rheumatic fever and rheumatic heart disease in national health policies and budgets, the paucity of data to enable targeting of prevention efforts, limited understanding of rheumatic fever and/or rheumatic heart disease in affected communities, and inaction on the social determinants of the disease and inequities in health [5]. In Bhutan we have communities where people prefer help from local healers than to visit health centres. In our case, the patient’s father initially refused our help and wanted to go to local healer but after explaining the disease condition to him, he agreed and remained with us.