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.