Sydenham' s Chorea (Chorea Minor, " St. Virus' Dance") This neurologic syndrome occurs after a latent peri-od that is variable but on the average longer than that associat-ed with the other manifestations of ARF. If frequently occurs in "pure" form, either unaccompanied by other major manifes-tations or, after a latent period of several months, at a time when all other evidence of acute rheumatic activity has subsid-ed. Chorea is characterized by rapid, purposeless, involuntary movements, most noticeable in the extremities and face. The arms and legs flail about in erratic, jerky, incoordinated move-ments that may sometimes be unilateral (hemichorea). Facial tics, grimaces, grins, and contortions are evident. The speech is usually slurred or jerky. The tongue, when protruded, re-tracts involuntarily, while asynchronous contractions of lingual muscles produce a "bag of worms" appearance. The involun-tary motions disappear during sleep and may be partially sup-peressed by rest, sedation, or volition.

Patients with chorea display generalized muscle weakness and an inability to maintain a tetanic muscle contraction. Thus. when the patient is asked to squeeze the examiner's fin-gets, a squeezing and relaxing motion occurs that has been de-scribed as "'milkmaid's grip. " The knee jerk may have a pen-dular quality. There is no cranial nerve or pyramidal involve-ment, and sensory modalities are unaffected. The electroen-cephalogram may display abnormal slow wave activity.

Emotional lability is characteristic of Sydenham' s chorea and often may precede other neurologic manifestations, leaving teachers and parents puzzled over apparently inexplicable per-sonality changes.

Subcutaneous Nodules These are firm, painless subcuta-neous lesions that vary in size from a few millimeters to ap-proximately 2 cm. The skin overlying them is freely movable and is not inflamed. The lesions tend to occur in cross over bony surfaces or prominences and over tendons. Sites of predilection include the extensor surfaces of elbows, knees, and wrists; the occiput; and spinous processes of the thoracic and lumbar vertebrae. Nodules are virtually never the sole ma-jot manifestation of ARF; they almost always appear in associ- ation with carditis, and the cardiac involvement in such cases tends to be clinically severe. Nodules ordinarily do not appear until at least 3 weeks after the onset of an attack, usually last-ing 1 to 2 weeks. They may appear in repeated crops in pa-tients with protracted carditis. Similar nodules may be seen in systemic lupus erythematosus and in rheumatoid arthritis. Subcutaneous nodules in the latter disease are larger and more persistent than those in rheumatic fever.

grythema Marginatum The rash begins as an erythema-tous macule or papule, which then extends outward, while the skin in the center returns to normal. Adjacent lesions coalesce, forming circinate or serpiginaous patterns. The lesions coa-lesce, forming circinate or serpiginous patterns. The lesions are neither pruritic nor indurated, and they blanch on pres-sure. They vary greatly in size and appear mostly upon the trunk and proximal extremities, sparing the face. Erythema marginatum may be raised or flat; the latter was termed ery-thema annulare in the older literature. The lesions are evanes-cent, migrating from place to place, at times changing before the observer' s eyes, and leaving no residual scarring. The ery-thema may be brought out by the application of heat. Individ-ual lesions may come and go in minutes to hours, but the pro-cess may go intermittently for weeks to months uninfluenced by artti-inflammatory therapy; its persistence is not necessarily an advers progenositc sign. In the great majority of cases, ery-therna marginatum is accompanied by carditis; it also tends to be associated with subcutaneous nodules.

Laboratory Findings No specific laboratory test is diag-nostic of ARF. Usually there is a leukocytosis with an increase in the proportion of polymorphonuclear leukocytes. A mild to moderate normocytic normochromic anemia is the rule. In some patients the serum aspartate aminotransferase (AST) level is elevated. Evidence of acute inflammation is prominent, including readily detectable quantities of C-reactive protein in the blood and elevation of the erythrocyte sedimentation rate. An exception is "pure" chorea, which may appear long after indices of inflammation have returned to normal.

The urine may contain protein, white cells, and red cells. Biopsy studies have revealed a variety of renal abnormalities, but the classic proliferative glomerular abnormalities that char-acterize poststreptococcal acute glomerulonephritis occur quite rarely in ARF. Electrocardiographic and radiographic studies may reveal evidence of rhythm disturbances, pericarditis, or congesnve heart failure. Echocardiography may document my-ocardial and valvular dysfunction and pericardial effusion.

The major laboratory contribution to the workup of ARF is the documentation of recent group A streptococcal infection. Throat culture should always be performed but is positive in only a minority of cases. This is perhaps due to the time lapse of several weeks between the onset of the pharyngeal infection and the throat culture. The serum titer of ASO is elevatgd in 80 per cent or more of ARF patients. If two streptococcal anti-body tests, e.g., ASO plus either anti-DNase B or anti-hyaluronidase, are performed, an elevated titer of at least one will be found in 90 per cent of ARF patients. A b.attery d three tests establishes the presence of recent, immunologically significant streptococcal infection in more than 95 per cent of individuals experiencing an acute rheumatic attack. The defi-nition of an "elevated" titer varies, depending upon the test employed, age of the patient, and geographic locale. ASO titers greater than 200 to 250 Todd units per milliliter are gen-erally considered elevated. At times, serial sampling may de-tect a rising titer of streptococcal antibodies in patients seen early in the course of a rheumatic attack.

Course and Prognosis The average duration of an un-treated attack of ARF is approximatelyo3 months. The dura-tion tends to be longer, up to 6 months, in patients with se-vere carditis. Fewer than 5 per cent of patients have continu-ing rheumatic activity for longer than 6 months. In a few of these the disease is limited to chorea and is otherwise benign. Other patients exhibit evidence of persistent inflammatory ac-tivity, including arthritis, carditis, and subcutaneous nodules. "Chronic rheumatic fever" occurs more frequently in patients who have had one or more previous attacks; cardiac involvement in chronic rheumatic fever tends to be frequent and severe.

Death from intractable myocarditis during the acute phase of ARF is now very rare. Once the acute attack has subsided, the only long-term sequel is that of rheumatic heart disease, manifested primarily by insufficiency and/or stenosis of the mitral and aortic valves. The prognosis from a cardiac stand-point is very much dependent upon the clinical findings at the time the patient is first seen. In one large study, for example, 347 patients were examined during an acute rheumatic attack and again 10 years later. Among patients who had been free of carditis during their acute attack, only 6 per cent had a residu-al heart disease on followup. Patients with no pre-existing heart disease and with mild carditis during their acute attack (i. e. , apical systolic murmur without pericarditis or heart fail-ure) had relatively good prognosis in that only approximately 30 per cent had heart murmurs 10 years later. About 40 per cent of subjects with apical or basal diastolic murmurs and 70 per cent of subiects with failure and/or pericarditis during their acute attacks had residual rheumatic heart disease. The prog-nosis was worse in patients with preexisting heart disease and in those who had experienced recurrent attacks of ARF in the 10-year interval.

The data cited above indicate that patients who do not de-velop carditis during an acute attack and are protected from ARF recurrences are most unlikely to suffer from rheumatic heart disease. The patient with "pure" chorea represents an exception to this rule. A significant proportion of such patients who have no evidence of carditis when first examined may de-velop rheumatic valvular disease on prolonged follow-up. Al-though the explanation for this phenomenon is unknown, it is conceivable that in view of the long latent period associated with chorea, signs of carditis might have been present earlier but subsided by the time the neurologic abnormality became evident.

Diagnosis Although ARF is readily recognized in the in-dividual who presents with multiple major manifestation or in epidemic circumstances, at other times the disease may be ex-traordinarily difficult to diagnose with confidence. This is be-cause of the variability of its clinical presentation, the frequen-

TABLE 9 Jones Criteria(Revised) for Guidance

In The Diagnosis of Rheumatic Fever

The diagnostic criteria of T. Duckett Jones, as subse-quently modified by a committee of the American Heart Asso-ciation, attempt to minimize overdiagnosis and underdiagnosis (Table 9). Two major manifestations, or one major and two minor manifestations, indicate a high probability of ARF, pro-vided that there is supporting evidence of recent streptococcal infection. Although a positive throat culture for group A strep-tococci technically satisfies this requirement, streptococcal car-riage rates of 15 per cent are not uncommon among school-aged children during the fall and winter. Elevated titers of antibod-ies to streptococcal extracellular products, although not diag-nostic of ARF, do indicate a recent, immun01ogically signifi-cant streptococcal infection. Conversely, if a battery of strep-tococcal antibody tests fails to reveal any evidence of recent in-fection, the diagnosis of ARF must be considered unlikely. This statement does not necessarily hold true in patients whose only rheumatic manifestation is Sydenham's chorea. Because of the long latent period associated with chorea, previously ele-vated antibody titers may have declined to normal.

The modified Jones criteria are, of course, only guide-lines. They are most difficult to apply confidently when pol-yarthritis is the single major manifestation. Under such cir-cumstances, serious consideration must be given to various other entities, including rheumatoid arthritis, Still's disease, viral arthritides (e. g. , rubella, hepatitis B), the early prepur-puric phase of Henoch-Schonlein purpura, and septic arthritis, including gonococcal arthritis.

Serum sickness is frequently a serious consideration, par-ticularly if the patient has received penicillin or other antibi-otics for a preceding respiratory infection. Systemic Iupus ery-thematosus, sickle cell hemoglobinopathies, and infective en-docarditis may involve the joints and the heart. Other differen-tial diagnostic considerations include congenital heart lesions, viral and idiopathic forms of myocarditis and peicarditis, and functional heart murmurs. Nonfamilial forms of chorea have been described in systemic lupus erythematosus and rarely in association with the use of birth control pills, It remains un-certain how often episodes of chorea occurring buring pregnan-cy ("chorea gravidarum") represent attacks of rheumatic fever, Other disorders that may at times be confused with ARF are gout, sarcoidosis, Hodgkin' s disease, and acute leukemia.

Following an episode of acute streptococcal pharyngitis, a small proportion of patients may experience persistent symp-toms of malaise, arthralgia, low-grade fever, and lym-phadenopathy plus laboratory evidence of mild inflammatioin. It is difficult to classify such cases, but the affected individuals do not meet the criteria for diagnosis of ARF and, moreover, do not appear to be normal risk for the delayed cardiac sequelae of ARF.

Treatment Antibiotice neither modify the course of a rheumatic attack nor influence the subsequent development of carditis. Nevertheless, it is conventional to give a course of an-tibiotics designed to eradicate and rheumatogenic group A streptococci remaining in the tonsils and pharynx, at least in part to prevent spread of the organism to close contacts. The recommended regimens are those conventionally used for treat-ment of acute streptococcal pharyngitis. Benzathine penicillin G is preferred in the non-penicillin patient. Following comple-tion of this therapy, continuous antistreptococcal prophylaxis should commence.

Treatment with anti-inflammatory agents is effective is suppressing many of the signs and symptoms of ARF. These agents do not "cure" the disease, nor do they prevent the sub- sequent evolution of rheumatic heart disease. They should be avoided in very mild or equivocal cases because, by suppressing the clinical manifestations, they may obscure the diagnosis. The two drugs most widely used are aspirin and corticos-teroids. The former is used in patients with acute polyarthri-tis, provided that carditis is either absent or mild and there is no evidence of congestive heart failure. Aspirin is very effective in decreasing fever, toxicity, and joint inflammation. It should be given in a dosage of 90 to 100 mg per kilogram per day in children and 6 to 8 g per day in adults. This is administered in equally divided doses, every 4hours for the first 24 to 36 hours, thereafter it may be given in four doses during waking hours. A salicylate level of 25 mg per deciliter is satisfactory. The incidence of nausea and vomiting may be minimized by starting somewhat below the optimal dosage level and gradual-ly increasing over a few days. The patient should be observed for evidence of significant gastrointestinal bleeding and for signs and symptoms of salicvlism. After 2 weeks, the dosage is reduced to 60 to 70 mg per kilogram per day for an additional 6 weeks. These dosage schedules represent general guidelines only. The precise aspirin dose must be determined by the pa-tient's clinical response, blood salicylate levels, and tolerance of the drug.

Corticosteroids are generally reserved for patients who have severe carditis manifested by congesive heart failure, who are unable to tolerate large doses of salicylates, or whose signs and symptoms are inadequately suppressed by aspirin. As with aspirin, the dosage must be individualized. Prednisone, 40 to 60 mg per day in divided doses, may be used initially, after 2 to 3 weeks it should be withdrawn slowly over an additional 3 weeks period. In cases of fulminating carditis with profound heart failure, intravenous corticosteroids may be employed. Aspirin should be administered for a month after discontinua-tion of prednisone. As is the case for other patients receiving corticosteroids, the physician should be alert to problems such as gastrointesinal bleeding, sodium and water retention, and impairment of glucose tolerance. Suppression of the pituitary-adrenal axis or of the host immune system is a potential prob-lem but not ordinarily a major one during this relatively short course of treatment. The orle of nonsteroidal anti-inflammato-ry agents in management of ARF remains to be defined.

Following cessation of anti-inflammatory therapy, clinical or laboratory evidence of ARF may reappear. Such therapeutic "rebounds" occur more frequently after corticosteroid therapy than after treatment with aspirin. They may be minimized by prolonging salicylate therapy for 9 to 12 weeks and, when cor-ticosteroids have been required, by continuing aspirin use for a month after corticosteroids have been discontinued.

Congestive heart failure is managed by the usual measures of bed rest, sodium restriction, diuretics, and if necessary, oxygen and digitalis. The potential risk of digitalis-induced ar-rhythmias in the patient with active myocarditis must be borne in mind.

All patients should be kept at bed-chair rest for the first 3 weeks of illness, during which time carditis will usually mani-fest itself if it is destined to appear. Bathroom priileges may be allowed unless arthritis or chorea makes this infeasible or un-less frank heart failure supervenes. Subsequently the level of physical activity should be guided by the patient's clinical sta-tus, primarily by the presence and activity of rheumatic cardi-tis, Patients with congestive heart failure should be kept at rest until compensation has been achieved. Patients with Sydenham's chorea require a quiet environment, and sedatives such as phenobarbital may be helpful.

Once the acute attack has subsided completely, the pa-tient's subsequent level of physical activity is dependent upon cardiac status. Patients without residual heart disease may re-sume full and unrestricted activity. It is important that the pa-tient not be subjected to unwarranted invalidism, either be-cause of his or her own inaccurate perceptions of the nature of the rheumatic process or because of those of parents, or em-ployers.

Prevention "Primary prevention" of ARF consists of ac-curate diagnosis and appropriate treatment of streptococcal sore throat. Although straightforward in theory, primary preven-tion is often frustratingly difficult to achieve. In many of the densely populated, indigent communities in which the risk of ARF is greatest, children with self-limited illnesses such as sore throats may never come to medical attention, and throat culture services are usually unavailable to aid in diagnosis. Moreover, in one third or more of cases, ARF may arise aftra clinically inapparent streptococcal infection.

Perhaps the most effective strategy for avoiding the mor-tality and chronic cardiac disability associated with ARF is that of "secondary prevention. " This strategy focuses upon the group of persons who have already suffered a rheumatic attack and who are inordinately susceptible to a recurrence following an immunologically significant streptococcal upper respiratory infection. Recurrent attacks tend to be mimetic in nature, so that patients who have suffered carditis with their previous at-

tack are likley to have repetitive cardiac involvement and pro-gressive cardiac dam.

1 2

• How to contact us
• How to order our products
• How to pay
• What's our service after sale