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The protean manifestations of this condition were well described in the early part of the last century with a set of diagnostic criteria proposed by Duckett Jones in 194428 and subsequently modified and updated in 1992 and 2002.29,30,31,32,33 These revisions increased specificity but decreased sensitivity, particularly in endemic populations with potential missed diagnoses and failure to provide secondary prophylaxis to deserving patients.34 Local guidelines have been adapted to the Jones criteria to reflect the pattern of disease in high-prevalence regions, local evidence, and current best practice, particularly relating to contemporary echocardiographic techniques.35,36 The Jones criteria recently underwent an extensive revision.37 This revision has aligned itself with international guidelines by defining high-risk populations, recognizing the variability in disease burden and presentation, categorizing recommendations according to the favored Classification of Recommendations and Level of Evidence categories, and reflecting the era of Doppler echocardiography38 (Table 46–1).
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The disease usually has an acute febrile onset and presents with variable combinations of major and minor manifestations. The diagnosis of ARF is made when the patient develops two major manifestations, or one major manifestation and at least two minor manifestations; in addition, evidence of preceding infection with GAS must be demonstrated using streptococcal serology. The exceptions are patients who present with chorea or indolent carditis, because these manifestations may only become apparent months after the inciting streptococcal infection so that additional manifestations may not be present and streptococcal serology testing may be normal.39
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Carditis is the single most important component of the disease in determining prognosis. The carditis of ARF occurs in > 50% of patients and is characterised predominantly by valvulitis of the mitral valve (mitral regurgitation) and, less frequently, the aortic valve (aortic regurgitation).40,41,42 Pathologically, ARF produces a pancarditis, with involvement of the pericardium, myocardium, and endocardium. Clinical manifestations may vary widely and range from subclinical to life-threatening heart failure. Pericarditis may manifest with typical pericardial pain and a friction rub. Auscultation may reveal new murmurs or changing murmurs. Stenotic lesions are uncommon in the early stages of the disease, but a transient apical mid-diastolic murmur (Carey-Coombs) may occur in association with the murmur of mitral regurgitation. This murmur occurs in patients with mitral valvulitis due to ARF. It is a short, mid-diastolic rumble best heard at the apex, which disappears as the valvulitis improves. It can be distinguished from the diastolic murmur of mitral stenosis by the absence of an opening snap before the murmur. The murmur is caused by increased blood flow across a thickened mitral valve.43
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Atrioventricular conduction delay, resulting in a prolonged PR interval, is an important and helpful diagnostic clue. Recent data strongly substantiates the importance of subclinical carditis based on Doppler echocardiography.42,44,45,46,47 Hence, echocardiographic evaluation for all patients with suspicion of ARF is recommended in the 2015 Jones criteria, and both clinical and subclinical carditis fulfil a major criterion, even in the absence of classical auscultatory findings.48 Subclinical carditis of the mitral or aortic valves requires all four criteria to be met in order to fulfil the major criterion. Additional morphologic features may also be present.
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Arthritis and Arthralgia
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The conventional description is of a polyarthritis affecting large joints, with the lower limb joints involved first, and involvement of each joint overlapping giving the impression that the process “flits.” The arthritis of ARF responds promptly to anti-inflammatories, and thus, the classical presentation may be uncommon where medication with nonsteroidal anti-inflammatory drugs before the diagnosis is considered or confirmed.
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The differential diagnosis of polyarticular arthritis in children and adolescents is wide (Table 46–2).49 Monoarticular arthritis is increasingly recognized to be important in populations where ARF is common. The arthritis of ARF presents a difficult diagnostic challenge, especially in the patient presenting with an initial monoarthritis.50 The arthritis of ARF is highly responsive to anti-inflammatory drugs (both aspirin and nonsteroidal anti-inflammatory drugs), and if the patient does not respond within 48 to 72 hours, alternate diagnoses should be considered. Poststreptococcal reactive arthritis is diagnosed in patients who have an arthritis that is not typical of ARF but who recently had streptococcal infection. This condition is said to occur after a shorter latent period than ARF, responds less well to anti-inflammatories, and may be associated with renal manifestations, and evidence of carditis is usually not seen. The distinction between post-streptococcal reactive arthritis and ARF is unclear, and many would recommend that a diagnosis of post-streptococcal reactive arthritis not be made in populations in which ARF is common.49 Even if the diagnosis is considered, it is appropriate to offer a period of secondary penicillin prophylaxis, as for episodes of ARF in such populations.
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Sydenham chorea may be associated with other manifestations of ARF but may also be the sole expression of the disease. It is a neurologic disorder characterized by involuntary, purposeless, rapid, and abrupt movements associated with muscular weakness and emotional lability. Chorea occurs in up to 30% of cases of ARF. The abnormal movements disappear during sleep.
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Mild chorea may best be demonstrated by asking the patient to squeeze the examiner’s hand. This results in repetitive irregular squeezes labeled as the milking sign. Emotional lability manifests in personality changes, with inappropriate behavior, restlessness, and outbursts of anger or crying.51
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Erythema marginatum is a nonitchy, evanescent rash that is pink or slightly red and that affects the trunk predominantly. The rash extends centrifugally, and the skin in the center returns toward normal. The rash may be fleeting and disappear within hours. It may be brought out by a warm bath or shower. It is reported to be found in only 4% to 15% of cases and may be difficult to detect in dark-skinned patients.
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These nodules generally appear later in the course of the disease after several weeks of illness and are seen most commonly in patients with carditis. They are firm and painless; the overlying skin is not inflamed and may vary in size from a few millimeters to several centimeters. They are most commonly located over bony surfaces or tendons such as elbows, wrists, knees, occiput, and spinous processes of the vertebrae. These occur in less than 10% of cases of ARF.