The classical presentation is that of migratory and asymmetric polyarthritis involving large joints, especially in the legs, and than migrating to other joints with some overlapping of joints involvement. It occurs in approximately 75 % of the cases and should be differentiated from arthralgia (no inflammatory signs). When associated with carditis, there seems to be an inverse correlation between the severity of the two findings [1]. It responds rapidly to anti-inflammatories and thus, self-medication may mask the typical presentation.

It is the most serious manifestation of the disease, and the only one that leaves sequelae. Clinically, it is present in 40–70 % of the cases, however the percentage is much higher when the diagnosis is made by echocardiography [18, 19]. Although there is a pancarditis (endocardial, myocardial and pericardial involvement), valve lesions are the ones responsible for the clinical presentation and prognosis. Myocarditis can be diagnosed by histology, but it does not cause HF and systolic function is usually preserved at the initial presentation. Pericardium involvement is not common, does not happen in isolation and does not lead to constriction, but the presence of a pericardial effusion may help confirm the diagnosis [20].

The most common valve lesion is MR and its pan-systolic murmur does not indicate permanent lesion. Aortic regurgitation (AR) is less common and stenotic lesions do not happen in the early stage of the disease. Tricuspid regurgitation may occur in acute carditis secondary to pulmonary hypertension.

The severity of carditis can vary from SCC to a fulminant form. In SCC, cardiovascular exam, X Ray and ECG are normal (except for a prolonged PR). Doppler echocardiogram is essential for its diagnosis, as it can detect pathological mild MR and/or AR [21]. Mild carditis is present when there is tachycardia disproportional to the degree of fever, diminished S1 and MR systolic murmur. Chest X Ray and ECG are normal (except for a prolonged PR), but Doppler echocardiogram shows mild or moderate regurgitations and a normal-sized left ventricle (LV). In the moderate form, cardiovascular exam is abnormal, with signs of incipient HF, abnormal chest X Ray and ECG, and more severe regurgitations in the echocardiogram, with enlarged LV. Finally, in the severe form, cardiovascular signs are more important, the patient presents in HF, with murmurs related to more severe degrees of regurgitation, arrhythmias, pericarditis, and several abnormalities on the ECG, chest X Ray and Doppler echocardiogram [20].

Sydenham’s chorea may occur in association with other manifestations, but it may also be the sole expression. It is a neurological disorder characterized by rapid and involuntary movements, which are more common during stress and cease during sleep. Its incidence varies from 5 to 36 % and it occurs predominantly in female children and adolescents [20].

It is a rare skin manifestation (4–15 % of the patients), generally occurring at the beginning of the disease. It is usually associated with carditis, but not with its severity, being characterized by a non-itchy pink-red lesion, which predominantly affects the trunk and spares the face. It may disappear within hours and it is difficult to detect in dark-skin patients [20].

They are firm and painless, varying greatly in size, and representing a rare manifestation (2–5 %) of the disease. The overlying skin is not inflamed and they are usually located over bones surfaces or tendons and best detected by palpation and not inspection. Their presence is strongly associated with severe carditis [20].

They are unspecific and only when associated with major criteria and evidence of previous GAS infection help to establish the diagnosis. Fever and tachycardia out of proportion to the fever are usually present. Fever is usually of low grade when there is carditis without arthritis, and absent in isolated chorea [20].

After recovery from the initial episode, 72 % of patients will develop valve heart diseases [18]. Recurrence of the disease can lead to progression of valve lesions with all its consequences, as HF, atrial fibrillation, stroke, infective endocarditis and pregnancy-related complications. With progression of the disease, cardiac surgery becomes mandatory and, if not performed, premature death from RHD and its complications is frequent, with some countries presenting the unacceptable mean age of death <25 years [22, 23]. Otherwise, patients under regular secondary prophylaxis may present recovery on the severity of valve lesions.

ECG is unspecific, since it can be normal in the presence of carditis. A prolonged PR interval (minor sign) can be present in the absence of carditis. Sinus tachycardia, ST-T abnormalities, low QRS and T amplitude in the frontal leads can be present [20].

Although echocardiogram has been shown to be much more sensitive in the diagnosis of rheumatic lesions, screening by echocardiography is not always feasible, especially in low-income countries, where the disease is usually more prevalent. Besides, physiological regurgitations in normal individual can be interpreted as secondary to RHD. To avoid this misclassification, regurgitation should be considered abnormal only in the presence of morphological valve abnormalities [21] (Table 3).

Since secondary prevention can avoid adverse outcomes, early echocardiographic-based diagnosis of valve lesions by active surveillance strategies has been shown in several countries to be of major importance [24–27].

MR is the most frequent lesion in ARF, being present in up to 94 % of the cases. Valve thickening and focal nodules in the distal portion of the leaflets are frequent and disappear in the follow-up [28]. AR is not a frequent lesion in ARF, but in males it can occasionally be an isolated lesion. Stenosis is a late finding. LV dilation may be present and both cardiomegaly and valve regurgitation can disappear. Systolic function is usually preserved and HF, when present, is considered nowadays to occur due to valve lesion and not to myocardium involvement.

In patients with chronic RHD, recurrence is always associated with carditis, which can be expressed as pericarditis, new or worsening of a pre-existing valve regurgitation, increase in cardiac silhouette and HF. Size and function of cardiac chambers, left valve abnormalities (stenosis and regurgitation), tricuspid lesion (much less frequent) and associated pulmonary hypertension can all be adequately detected by echocardiography in RHD.

Treatment aims to suppress the acute inflammatory process, minimizing clinical repercussions on the heart, joints and central nervous system, in addition to eradicating GAS infection and promoting relief of main symptoms [20, 29].