A 23-yr-old male presented with large joint arthritis, petechial skin rash, and cardiac murmurs- 25th May 2022

The history of the sore throat followed by large joint arthritis, petechial skin rash, and cardiac murmurs were typically suggestive of acute rheumatic fever (ARF). However, his age and the presence of these symptoms, and the persisting additive pattern were atypical of ARF. The other possibilities to be considered are post-streptococcal reactive arthritis, bacterial endocarditis in pre-existing rheumatic heart disease, or other inflammatory arthritis like reactive or post viral arthritis, rheumatoid arthritis, or connective tissue disease with a pre-existing cardiac condition.

The Jones criteria¹ followed for the diagnosis of ARF are given below:

Evidence of streptococcal infection

• Throat culture positive for the bacteria
• Positive rapid antigen detection test results
• Elevated antistreptolysin O (ASLO) titer
• Scarlet fever

Diagnosis requires 2 major or 1 major and 2 minor criteria and evidence of streptococcal infection or chorea alone.

The patient fulfilled two major criteria and has elevated ASLO as evidence of streptococcal infection with a typical sore throat history, two weeks before the onset of arthritis. He had no cardiac symptoms, but examination revealed murmurs. The ECHO showed the valvular involvement. In this case, the patient has both the aortic valve and mitral disease suggestive of ARF. In a clinical scenario where only, the mitral valve shows mild regurgitation, it is difficult to establish whether the mitral disease is due to mitral valve prolapse (MVP) or rheumatic diseases. There are established echocardiographic criteria that are used to differentiate MVP from true rheumatic disease. But in the present case, involvement of both aortic and mitral valves was reported. However, there are published criteria to detect early mitral valve disease in ARF.

Isolated arthritis is the presenting symptom in 14­.42% of the patients.^2,3,4 The patient may be suffering from silent carditis without an antecedent history of sore throat, or he would have ignored the symptom. ⁵

The diagnostic dilemma in the present patient is whether the current episode is a relapse of rheumatic fever or a fresh episode of ARF. The patient had both aortic and mitral regurgitations with thickened valves. He could not recollect having arthritis or any symptoms suggestive of cardiac involvement. He had no symptoms related to cardiovascular disease and had not undergone any screening for cardiac functioning. In a series of 125 patients with mitral stenosis, only 54 patients had been reported with a definite history attributable to ARF.⁶ To categorize the condition as relapse by criteria, history of ARF, or rheumatic heart disease (RHD) with one major criterion is required. However, in this scenario, it is difficult to differentiate between relapse and recent-onset ARF. Further follow-up and resolution of valvular condition may help in retrospective differentiation, but it may be difficult to confirm. The incidence of silent rheumatic valvular disease was found to be 10 to 20 times higher upon using echocardiography for disease diagnosis.⁷ Considering all the possibilities, it could be concluded that the current episode could be a relapse rather than a fresh ARF. Even the first episode of ARF is rather rare in this age group.

Recent epidemiological data demonstrate a gradual decline in the incidence of ARF in India. The reported incidence of 5.3 in 1970-80 has dropped to below 1 per thousand, as per the survey conducted by ICMR among school children.⁸

The petechial rashes described in the patient are rather unusual for ARF. Erythema marginatum, the typical skin lesion of ARF is less commonly described in the Indian Series.⁶ The possibility of subacute bacterial endocarditis (SBE) should be suspected in the presence of petechial rashes, and arthritis with pre-existing valvular disease. The SBE on normal valves leading to regurgitation as well as asymptomatic mitral valve disease presenting with SBE is well described in the literature. The Dukes criteria (Table 3) with modifications are used currently to define infective endocarditis.⁹ However, in the current case, blood cultures were negative, and the echocardiography had no evidence of infective endocarditis. Transthoracic echocardiography was not done, since the suspicion of IE was less likely and microscopic urinalysis showed no RBC, normal Hb%, and absence of leucocytosis. Petechial rashes were minimum and inconsistent with that of SBE. The fever was moderate and reduced with NSAID treatment.

Table 3: Modified Dukes criteria

Elevated ASLO reflects streptococcal infection. Post-streptococcal reactive arthritis is a well-described entity, which does not have carditis as a manifestation and does not require penicillin prophylaxis.^10,11 Streptococcal infections may trigger an exacerbation of the existing rheumatological syndrome. Hence, it is crucial to carefully interpret elevated ASLO titer. Misinterpretation of elevated ASLO as ARF is common. On contrary, the epidemiological data suggest underrecognition of rheumatic fever in the community, especially rheumatic heart disease.

Other possible diagnoses could be reactive arthritis and rheumatoid arthritis. The involvement of the small joints of the hand is less commonly described in ARF. Hence a probability of RA can be considered. However, other findings do not suggest the diagnosis. The possibility of vasculitis is less common.

Course of treatment

The patient was diagnosed with ARF, and the treatment involved penicillin along with NSAID and a tapering dose of steroids. Within 2 days, the pain reduced, and the patient was mobile. He was prescribed penidure prophylaxis.

Learning Points

1. Careful clinical examination and echocardiographic evaluation are crucial in suspected cases, though the incidence of rheumatic fever is very rare.
2. There are established criteria for diagnosing carditis and they are useful in suspected cases to establish rheumatic carditis.
3. Elevated ASLO is not necessarily rheumatic fever; other possible diagnoses should be excluded.

References

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