Carditis Antiinflammatory drugs have been extensively employed in the treatment of carditis, showing a prompt effect in terms of cardiac symptoms (Table 54.4) Salicylates have been used for those with no or mild forms of carditis when there is no necessity for powerful suppression of inflammation Corticosteroids have been recommended for patients with at least moderately severe carditis, especially for those with congestive heart failure Prednisone is usually preferred as it produces less retention of sodium and loss of potassium The initial dose of prednisone is given for 2 to 3 weeks depending on the severity of the carditis After this period, a gradual tapering is required The dose should be slowly withdrawn, decreasing by one-fifth each week, which means a total duration of the treatment of about 8 to 12 weeks, coinciding with the duration of the acute episode Rebounds are sometimes associated with rapid withdrawal of corticosteroids Some authors have recommended an overlapping therapy with the aim to prevent rebounds, with the introduction of salicylates as the dose of corticosteroids is reduced The advantage of the use of high doses of methylprednisolone as an alternative treatment remains controversial It has most frequently been administered as a life-saving therapy for those with severe carditis The use of intravenous gamma globulin has been reported in patients with carditis, but more investigations are required to evaluate this therapy.190,192 Table 54.4 Treatment of Major Manifestations of Carditis Clinical Manifestation Severe carditis Moderate carditis Mild carditis Polyarthritis Chorea Treatment Schedulea Prednisoneb 2 mg/kg/day once daily Prednisone 1–2 mg/kg/day once daily or Aspirinc 75–100 mg/day divided into 4 doses Aspirin 75–100 mg/day divided into 4 doses Aspirin 75–100 mg/day divided into 4 doses or Naproxen 10–20 mg/kg/day Carbamazepine 4–10 mg/kg/day or Valproic acid 20–30 mg/kg/day or Haloperidold 2–6 mg/day aSee text for complementary information bMaximum dose is 80 mg/day cSerum level should not exceed 30 mg/dL d Maximum dose is 6 mg/day Supportive measures include the treatment of congestive heart failure and arrhythmias Congestive heart failure in rheumatic carditis is often controlled with bed rest and corticosteroids However, a detailed cardiac assessment including echocardiography is recommended for all patients in heart failure secondary to rheumatic carditis Diuretics and fluid restriction should be administered Angiotensin-converting enzyme inhibitors can be used in patients with significant valvar regurgitation and/or left ventricular dysfunction The lack of clinical improvement due to mechanical factors can be observed in patients with severe valvar lesions, mainly in those with recurrences, and sometimes the treatment is surgical Cardiac surgery is best deferred in the acute stage as the valve repair is difficult and less durable However, urgent surgery may be required due to very severe mitral and/or aortic regurgitation with heart failure or in those with a flail leaflet due to chordae tendineae rupture Repair of the valve is to be preferred over valve replacement in young patients.193 Arthritis Salicylates are the first-line treatment for patients with established arthritis but without carditis These agents usually have a dramatic effect on the articular inflammatory process, with a prompt improvement, commonly within 24 to 48 hours In this context, premature treatment can interfere in the characterization of the migratory pattern of the arthritis, thus making the diagnosis difficult Paracetamol may be used until confirmation of diagnosis The differential diagnosis with other diseases must be considered when the articular symptoms are not responsive to salicylates An effective and safer dose of aspirin for children usually produces a level of salicylate in the blood of not more than 20 mg, which is well below the toxic range (see Table 54.4) The full dose is given for the first 2 weeks and subsequently is gradually reduced to up to 60 mg/kg over the next 2 to 3 weeks A smaller or a larger dose can be used, depending on the clinical response to treatment, and may be maximized for those failing to respond to lower doses A higher dose, such as 100 mg/kg, is required by few patients If more massive doses of salicylates are given, toxicity can occur Rapid breathing is one of the early signs of toxicity Nausea, vomiting, tinnitus, lassitude, and occasionally delirium, convulsion, and coma can occur Naproxen is an alternative therapy for patients who are allergic to or intolerant of aspirin.1,194,195 It has similar analgesic potency as salicylate but does not have the risk of Reye syndrome, a rare complication of salicylate Other nonsteroidal antiinflammatory drugs have not been systematically used to establish their role Sydenham Chorea Isolated chorea is treated symptomatically The patients should be kept in a quiet environment to protect them from external stimulation and stress Mild chorea may not require any specific treatment Precautions must be taken to prevent accidents and, according to their condition, appropriate sedation can be helpful As most drugs used for chorea are not without side effects, treatment should be considered if symptoms affect the normal activities significantly or patient has risk of injury Valproic acid and carbamazepine are preferred over haloperidol as haloperidol has significant side effects.196–199 Carbamazepine should be used initially and valproic acid given for refractory cases (see Table 54.4) Antiinflammatory agents are usually not indicated because chorea often occurs after the resolution of the systemic inflammatory process The use of corticosteroids and immunoglobulin in patients with isolated Sydenham chorea is controversial.200–205 A short course of corticosteroids may be considered for severe refractory cases The need for the eradication of Streptococcus, followed by regular secondary prophylaxis, is as important in chorea as in any other rheumatic manifestation Due to the more recent observations regarding the neuropsychiatric disorders and persistent disabilities in some patients, a more aggressive treatment has been recommended, along with the need for closer vigilance in the period of follow-up.139–141,206,207 Rebounds Clinical or laboratory evidence of rebounds is infrequent They can be seen after cessation of the antiinflammatory treatment, and usually occur within 2 to 3 weeks Some patients show only laboratory abnormalities Those with clinical rebounds usually show arthralgia, fever, and occasionally arthritis, but severe cardiac manifestations may also occur Rebounds occur more often after corticosteroid therapy than salicylates Addition of salicylates to steroids at the