Advanced Cardiovascular Medicine Ashok Seth, Sameer Shrivastava, Upendra Kaul
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Rheumatic Fever: Time for Newer Recommendations1

Ashutosh Marwah
 
INTRODUCTION
Acute rheumatic fever (ARF) is a unique nonsuppurative complication of pharyngeal infection with group A streptococcus. It affects major joints, central nervous system, subcutaneous tissue, and the heart. All except cardiac effects are reversible without leaving any long-term sequelae. Cardiac manifestations are the cause of long-term morbidity and mortality in young adults and children.
 
BURDEN OF RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE IN INDIA
The true incidence of rheumatic heart disease (RHD) and rheumatic fever (RF) remains unknown in India. Our estimates are based on hospital admissions and small sample surveys. According to various authors, RF is endemic and remains one of the major causes of cardiovascular disease, accounting for nearly 25–45% of the acquired heart disease. We are also observing a decline in the overall incidence of RF similar to that in Western countries. Perhaps the decline is more in the urban population as compared to rural areas. Population-based surveys for prevalence are very few and scattered. In a study in rural Haryana, prevalence of RHD was found to be 2.2/1000 in 5–30 years old subjects.1 In a study of the urban population of Agra, Mathur et al. found RHD in 1.8/1000 in the same age group.2 Berry3 studied the urban population of Chandigarh and found RHD in 1.23/1000 male and 2.07/1000 in the female population of all age groups. The incidence has been quoted to be 0.5/1000 in a study conducted by Misra et al. in Uttar Pradesh on 1,18,212 school children 4–18 years in age.4
The ICMR has conducted three major surveys in school going children between the years 1970 and 2010. In the first study (1972–1975), 1,33,000 children were evaluated and the prevalence of RHD varied from 0.8 to 11/1000 (overall 5.3/1000). The second study (1984–1987) included 53,786 children and the prevalence ranged from 1.0 to 5.6/1000 (overall 2.9/1000). The third and the largest study included 1,76,904 school children with a prevalence varying from 0.13 to 1.5/1000 (overall 0.9/1000) in the 5–14 years age range.5 Though the data suggests the declining trends, various authors, however, have refuted this claim.
 
REVISED JONES CRITERIA FOR ACUTE RHEUMATIC FEVER69
Diagnosis of RF is based on Jones criteria, which were first published in 1944, and have been modified three times since then. The latest modification was published in 1992 and has remained unchanged. A definitive diagnosis requires that two major or one major and two minor criteria are satisfied, in addition to evidence of recent streptococcal infection.
 
Major Criteria
  • Carditis: All layers of cardiac tissue are affected (peri­cardium, epicardium, myocardium, endocardium). The patient may have a new or changing murmur, with mitral regurgitation being the most common followed by aortic insufficiency.
  • Polyarthritis: Migrating arthritis that typically affects the knees, ankles, elbows, and wrists. The joints are very painful and symptoms are very responsive to anti-inflammatory medicines.
  • Chorea: It is also known as Sydenham's chorea, or “St Vitus’ dance”. There are abrupt, purposeless movements. This may be the only manifestation of ARF and its presence is diagnostic. It may also include emotional disturbances and inappropriate behavior.
  • Erythema marginatum: A nonpruritic rash that commonly affects the trunk and proximal extremities, but spares the face. The rash typically migrates from central areas to periphery, and has well-defined borders.
  • Subcutaneous nodules: Usually located over bones or tendons, these nodules are painless and firm.
2
 
Minor Criteria
  • Fever
  • Arthralgia
  • Previous RF/RHD
  • Acute phase reactants: Leukocytosis, elevated erythro­sedimentation rate (ESR) and C-reactive protein (CRP)
  • Prolonged P-R interval on electrocardiogram (ECG).
 
EVIDENCE OF PRECEDING STREPTOCOCCAL INFECTION
Any one of the following is considered adequate evidence of infection:
  • Increased antistreptolysin O (ASO) or other streptococcal antibodies
  • Positive throat culture for group A b-hemolytic strepto­cocci
  • Positive rapid direct group A streptococci carbohydrate antigen test
  • Recent scarlet fever.
 
PROBLEMS IN DIAGNOSIS OF RHEUMATIC FEVER IN INDIAN SCENARIO USING JONES CRITERIA
Various authors have suggested that patients in India often do not present with all the manifestations of RF. In a study by Pereira et al., only 30% patients fulfilled the criteria for diagnosing ARF, requiring an evidence of group A streptococcal infection. When the essential criteria of previous streptococcal infection were disregarded, the number rose to 87.7%.10
There are several fallacies, which include:11,12
  • RF cannot be diagnosed, if carditis is the only manifes­tation of the disease, particularly of the recurrence.
  • Patients with subclinical carditis can often be missed on clinical evaluation.
  • Migratory polyarthritis that is major criteria for making diagnosis is often absent in our population. More than 50% patients do not give typical history. However, monoarthritis and arthralgias are more commonly seen in our population.
  • Erythema marginatum is often missed in our population owing to darker skin and transient nature of the manifestation.
  • Subcutaneous nodules also included in major criteria are seen on one-third cases of RF. However, when present they are almost always associated with carditis. Similar nodules may be found in adults with rheumatoid arthritis.
  • Further, in our population, there is higher incidence of carrier state for streptococci, elevation of ASO may not be specific for RF, and an elevated ASO titer is found in only in 75–80% of cases. An elevated ASO may be found in 20% nnormal population. A single low ASO titer does not exclude RF.
 
WHO GUIDELINES13
Based on Jones criteria WHO has come out with guidelines 2002–2003 to help in facilitating the diagnosis of the following groups:
 
Primary Episode of Rheumatic Fever and Recurrent Episode of Rheumatic Fever without RHD
  • Two major manifestations
  • One major and two minor manifestations
  • Plus evidence of preceding streptococcal infection.
 
Recurrent Episode of Rheumatic Fever with Established RHD
  • Two minor manifestations
  • Plus evidence of preceding streptococcal infection.
 
Rheumatic Chorea and Insidious Onset Carditis
Other manifestations or evidences of streptococcal infection are not required.
 
Chronic Valve Lesions of RHD in Patients Presenting for the First Time
Do not require other criteria to be diagnosed as having RHD.
 
Further WHO Guidelines
  1. Patients may present with polyarthritis (or with only polyarthralgia or monoarthritis) and with several (3 or more) other minor manifestations, together with evidence of recent group A streptococcal infection. Some of these cases may later turnout to be RF. It is prudent to consider them as cases of ‘probable rheumatic fever’ (once other diagnoses are excluded) and advise regular secondary prophylaxis. Such patients require close follow-up and regular examination of the heart. This cautious approach is particularly suitable for patients in vulnerable age groups in high incidence settings.
  2. 3Patients with previous RHD may not completely satisfy all criteria.
  3. Infective endocarditis and congenital heart disease should be excluded.
 
OTHER INVESTIGATIONS TO AID DIAGNOSIS OF RHEUMATIC FEVER
Since Jones criteria are insufficient in diagnosing RF, other modalities have been evaluated for making a definitive diagnosis. The three main contenders are myocardial biopsy, radionuclide scanning, and echocardiography. All three have been discussed here:
 
Role of Myocardial Biopsy for Diagnosis of RF14,15
Since carditis is essential component of cardiac involvement in RF, role of myocardial biopsy has been studied for diagnosing carditis. Myocardial biopsies from patients were compared with biopsies from patients with quiescent disease. The results demonstrated virtual absence of myocarditis (based on Dallas criteria). There was evidence of interstitial inflammation that ranged from perivascular mononuclear cellular infiltration to histiocytic aggregates and Aschoff nodule formation. Histiocytic aggregates and Aschoff nodules were identified in only 30% of patients. Aschoff nodules were seen in 40% of the endomyocardial biopsies taken from patients with pre-existing RHD and who developed a possible recurrence of rheumatic carditis with congestive heart failure (CHF).
These results suggested that myocardial biopsy did provide any additional diagnostic information in patients with first episode of RF and unexplained CHF in patients with established RHD, and who presented with only minor manifestations of RF and elevated ASO titers, would indicate a high probability of rheumatic carditis, and that an invasive test may not be needed for the diagnosis.
 
Role of Radionuclide Scanning1619
Gallium-67, radiolabeled leukocytes, and radiolabeled antimyosin antibody have all been used to image myocardial inflammation. However, the results of these studies have revealed that gallium-67 imaging has better diagnostic characteristics than antimyosin scintigraphy; and the results also confirmed that rheumatic carditis is predominantly infiltrative, rather than degenerative, in nature. Although radionuclide imaging has been used successfully to identify rheumatic carditis by noninvasive means, WHO guidelines clearly state that there is not enough experience with such methods to allow them to be used for the routine diagnosis of RF.
 
Echocardiography for Diagnosis of RF1922
Echocardiography gives excellent details of the structural abnormalities, and the Doppler allows the evaluation of functional abnormalities. Yet, echocardiography has not been included as a criterion in the diagnosis of RF. Many authors have reported a higher rate of detection of valve leak in patients with suspected carditis, when subjected to echocardiography rather than relying on auscul­tation alone.
Major advantage seems to be detection of subclinical car­ditis or so-called “echocarditis”. In such cases of subclinical rheumatic carditis, annular dilatation, leaflet prolapse, and elongation of the anterior mitral chordae were observed, indicating that the valve might have been sensitized or damaged. These patients may remain asymptomatic or develop audible murmur or progress to irreversible sequelae such a mitral stenosis.
Echocardiography also can help in the evaluation of valve structure and differentiate between rheumatic and nonrheumatic structural abnormalities of cardiac valves. According to WHO, echocardiography is definitely useful to detect left-sided valvular lesions. However, making the diagnosis of subclinical rheumatic carditis solely based on this modality remains controversial. Until the results of long-term encompassing prospective studies are available to substantiate the therapeutic and prognostic importance of subclinical rheumatic carditis, the addition of echocardiography to the Jones criteria cannot be justified.
 
DO WE REALLY NEED NEW GUIDELINES?23,24
Some of the highest documented rates of ARF and RHD in the world are found in Aboriginal Australians, Maoris, Pacific Islanders in New Zealand, and Pacific Island nations. The prevalence of RHD is also high in sub-Saharan Africa, Latin America, the Indian subcontinent, the Middle East, and Northern Africa.
Indian working group came out with guidelines on diagnosis and treatment of RF in 2008. These guidelines are based on Jones criteria; however, these guidelines have failed to address the needs of Indian population. Countries like Australia and New Zealand have come out with their own guidelines for high-risk population, enabling clinicians to identify cases with incomplete manifestations of the disease and provide appropriate treatment.
 
MAJOR CHANGES
  • The ability to diagnose a recurrence of ARF in a patient from a high-risk group who has only one major plus one minor manifestation, provided that other more likely diagnoses are excluded.
  • 4Fever can be considered a minor manifestation based on a reliable history (in the absence of documented temperature), if anti-inflammatory medication has already been administered.
  • Polyarthralgia and aseptic monoarthritis have been included as major criteria for high-risk population.
  • Subclinical carditis has also been included as major criteria for the high-risk population.
  • Monoarthralgia has been included as minor manifestation for the high-risk population.
A new category of probable RF has been created to include patients in whom the clinician strongly suspected RF, but all the Jones criteria could not be fulfilled or there was absence of evidence for group A streptococcal infection or serology.
In these patients, presence of subclinical carditis (i.e. evidence of valvulitis on echocardiogram, monoarthritis, and polyarthralgia) was also considered to be major manifestation of RF. Monoarthralgia and a documented history of fever have been included in minor criteria.
These cases have been categorized into two major subgroups according to the level of confidence with which the diagnosis is made.
  1. Highly suspected ARF
  2. Uncertain ARF
Patients falling under “Highly suspected group” are managed as ARF and are provided secondary prophylaxis for minimum of 10 years or till the age 21 years (whichever is longer).
Patients falling under “uncertain rheumatic fever group” are provided secondary prophylaxis for 12 months or until an alternative diagnosis is confirmed. These patients are reassessed, and if there is still no evidence of RHD, secondary prophylaxis is stopped. Patients are then advised to report back in case of any recurrence of symptoms. However, if after 1 year there is evidence of RF, then patient is shifted to “highly suspected group” and treated accordingly.
The guidelines clearly state that:
  • Patients presenting with monoarthritis should be considered to have septic arthritis until proven otherwise.
  • Patients presenting with polyarthritis or polyarthralgia should be thoroughly investigated for alternative diagnoses, including arboviral infections in regions where these diseases are prevalent.
 
CONCLUSION
The Jones criteria have been periodically modified and updated; the 1992 update is currently the most widely used and quoted version. Each change was made to improve the specificity of the criteria at the expense of sensitivity, largely in response to the falling incidence of ARF in the USA. As a result, the criteria may not be sensitive enough to pick up disease in high-incidence populations, where the consequences of underdiagnosis may be greater than those of over diagnosis. It is imperative that high-risk countries like India should modify the guidelines to suit the need of Indian people.
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