An attempt at a scholarly discourse on Rheumatic Fever inVellore, India

As with all things academic there comes a time in every great endeavor that one must publish something scholarly. Unfortunately for many of my readers who are not in the medical profession, that time is now. As part of our scholarship and as part of our elective, I must write a scholarly discussion of a tropical disease seen here in India. I have chosen to write about Rheumatic Fever and its devastating sequelae—Rheumatic Heart Disease. While Rheumatic Fever is not typically considered a tropical disease in the truest sense of the term, it is considered a disease of developing nations. As many developing nations lie near the equator, ipso facto, I will consider Rheumatic Fever a tropical disease.

For those of you living in the states with children--esp school aged children as Rheumatic fever is considered a childhood disease occurring at median age of 10-- let me calm your fears that every time your child has a sore throat your child may die of Rheumatic Fever. While at the turn of the last century, there were reportedly 10 cases out of 1000, today the incidence is much less maybe .05 out of 1000 according to estimates given by the World Health Organization. This decline is due to the discovery and use of penicillin and to the fears resulting in untold physician visits by parents like many of you. So relax…you are doing a great job parenting your child. (FYI—as a self professed hypochondriac—I am sure I will be the first parent in line at the doctor’s office.) However, unlike the US, India has not benefited as much from modern practice and Rheumatic Fever continues to claim the lives of many. Worldwide estimates suggest that there may be as many as 30 million children and young adults with chronic rheumatic heart disease and 90,000 die each year from its complications.

It is thought that Rheumatic Fever is an immunologic consequence of antibodies against the M antigenic domain of Group A streptococcus which cross react with antigens present on cardiac tissue. While the exact mechanism and pathogenesis continues to elude researchers, histological evidence suggests this is true. As some of the brightest (and perhaps the most gunner-ish) remember, histological specimens of verrucous lesions on cardiac valvular tissue reveal Aschoff bodies consisting of perivascular foci of eosinophilic collagen surrounded by lymphocytes, plasma cells, and macrophages suggesting its immunologic origin.

But before any carditis occurs, the group A beta hemolytic streptococcal infection must not only colonize the pharynx, but it must also infect. According to many, being colonized by the bacteria is not a sufficient condition nor does it warrant antimicrobial coverage. Once infected by the bug, the child will likely complain of a sore throat although it has been reported in literature that some infections leading to Rheumatic Fever began as asymptomatic infections. Once across the epithelial barrier of the oropharynx, the bacteria incubate for a period of 2-4 days followed by an acute inflammatory response of 3-5 days of sore throat of fever, malaise, sore throat, headache and elevated leukocyte count. Many bugs and viruses can cause sore throats so it is important to document that a Group A Strep infection has occured—the cause of the worry of Rheumatic Fever. To diagnose in the office and at this stage of the disease, one can order the rapid antigen detection test to determine presence of Group A strep. If the patient is presenting some time later with evidence of Syndenham’s chorea and no sore throat one can order anti-streptococcal antibodies such as antistreptolysin-O (ASO) or anti-DNase B as the levels of these antibodies peak during the clinical features of Rheumatic Fever and can signify a recent Group A Strep infection.

In an effort to aid in the diagnosis of Rheumatic Fever, the Jones Criteria was published stating major and minor criteria necessary for diagnosis. However, the diagnosis of Rheumatic Fever can not be made unless it can be documented that Group A Strep infection has occurred as determined by the aforementioned tests. 2 major or 1 major and 2 minor criteria must be present to fulfill the Jones criteria which states:

Major criteria include: carditis, polyarthritis, chorea, subcutaneous nodules, and erythema marginatum.

Minor criteria include: fever, arthralgia, prolonged PR interval on the electrocardiogram, elevated acute phase reactants (increased erythrocyte sedimentation rate [ESR]), presence of C-reactive protein, and leukocytosis.

Rheumatic fever is reported to only occur in .3-3% of cases of Group A Strep pharyngitis. When it occurs, it usually happens weeks after the sore throat has resolved. Penicillin shortens the course of the pharyngitis and prevents future sequalae of Rheumatic Fever and Rheumatic Heart Disease.

Since I am an aspiring cardiologist and love to listen to the sounds a beating heart makes as blood flows through its chambers and valves, I must write a few words about the cardiac manifestations of Rheumatic Fever. Pancarditis is the most serious and second most common complication of Rheumatic Fever (occurring in 50% of cases) according to an article by emedicine. Evidence of carditis includes dyspnea with mild to moderate pleuritic chest pain accompanied by a new murmur and tachycardia out of proportion to the fever. Pericarditis and its accompanying friction rub are also known to occur. Murmurs of acute rheumatic fever are thought to be from valve insufficiencies. Rheumatic fever most commonly affects the mitral valve but it can also affect the Aortic valve as I have seen here in India. Mitral valve regurgitation is appreciated as an apical pansystolic blowing murmur which radiates to the left axilla. During active carditis, one can also hear the Carey Coombs murmur which is an apical diastolic rumble often accompanying severe mitral valve insufficiency. In addition, it is also possible to hear the high pitched, blowing, decrescendo murmur of aortic regurgitation heard during diasystole as well.

I was initially instructed to write only 1.5 pages. I included the details of the heart sounds not because I expect that many of you are at all interested in cardiology, but rather because I want you to appreciate the workings of our heart and the symphony of sounds one can hear. While in India, I have heard both systolic and diastolic murmurs in the same patient. These murmurs are quite rare in the states and it is my privilege to be in India and my blessing to meet these patients.

Finally, I would like to write a few words about the impact of Rheumatic Fever on the lives of people in Vellore. Rheumatic fever leading to Rheumatic Heart Disease (the scarring of the mitral and sometimes the aortic valves) is a great burden to this population. Chronic Rheumatic Heart Disease results in cardiomegaly with left atrial enlargement further resulting in atrial fibrillation followed by exacerbations of congestive heart failure. Over time and after bouts of re-infection of the damaged valves, patients will become so sick and that they will need corrective surgery and valve replacement which so many of these people can not afford. Just last week I met a 24 year old woman with such severe mitral valve insufficiency from Rheumatic Fever that she would die without surgery. Unfortunately, she had no money to afford surgery. She will likely die at a young age leaving her children without a mother. If only she could have received a 10 day course of penicillin when she was initially infected--a simple cure not given in the complicated world of poverty. It makes me sad to think if only she had been born in another place she might have had a different life. She is only one of many individuals I have met suffering from Rheumatic Heart Disease.

There is more to write…such as the treatment of congestive heart failure in acute rheumatic fever…the prophylaxis of recurrent infections of damaged valves… but I trust those of you needing to know this information can review these protocols online. I hope you have enjoyed this discussion and found it somewhat useful. Thank you for reading.

*** with reference of emedicine's article on Rheumatic Fever