Acute rheumatic fever

ARF is a multisystem collagen vascular disease that follows untreated or undetected group A streptococcal pharyngitis in <1% of persons. It is seen most commonly in children ages 5 to 15 and is associated with a genetic predisposition. It is more common in developing countries. There also appear to be strains of S. pyogenes more likely to be implicated in this condition (see Table 154.1).

The diagnosis of ARF is made clinically and is based on the modified Jones criteria (Table 154.2). The presence of two major or one major and at least two minor criteria suggests the diagnosis. Recent infection with S. pyogenes also must be suggested by either isolation of the organism from the throat or serologic evidence in the form of elevation of antistreptolysin O, antihyaluronidase, or antideoxyribonuclease B titers. The exception to this rule is chorea, which becomes manifest 2 to 6 months after infection, by which time evidence of a recent streptococcal infection may be lacking.

The most common clinical manifestations of ARF are carditis and arthritis. The former usually presents as a significant murmur, most commonly mitral insufficiency. Both myocarditis and pericarditis may accompany this valvulitis. It is the only manifestation that may result in residual disease. The arthritis is a migratory polyarthritis that generally involves the medium-size joints (elbows, wrists, ankles, and knees). Pain is often striking. Another characteristic finding is the dramatic response of the arthritis to salicylate therapy. Chorea known as Sydenham chorea or St. Vitus’ dance usually occurs as an isolated, often subtle, neurologic disorder with behavioral aspects, particularly emotional lability. Erythema marginatum and subcutaneous nodules are rarely seen. The strongest diagnoses of ARF are based on carditis or chorea. The weakest is based on arthritis as a single major manifestation with two minor criteria.

The term PANDAS (pediatric autoimmune neuropsychiatric disorder associated with group A Streptococcus) has been used to refer to a group of neuropsychiatric or behavioral disorders, particularly obsessive-compulsive disorder (OCD), Tourette’s syndrome, and tic disorder, with a possible relationship to group A streptococcal infections, and, perhaps, related pathologically to Sydenham chorea. Swedo and colleagues have proposed an autoimmune pathogenesis for these disorders, although this is controversial. Suggested diagnostic criteria include the presence of OCD or a tic disorder; pediatric onset; abrupt onset of symptoms or a course characterized by dramatic exacerbations of symptoms; a temporal association with group A streptococcal infection; and abnormal results of neurologic examination, such as choreiform movements, motor hyperactivity, and tics. Extensive investigation of its epidemiology, diagnosis, and treatment as well as its relationship to ARF are still underway.

Prevention

Primary prevention of ARF requires the proper diagnosis and treatment of S. pyogenes

Related posts:

Peritonitis Infectious complications in the injection and non-injection drug user Intracranial suppuration Syphilis and other treponematoses Hantavirus cardiopulmonary syndrome in the Americas Esophageal infections

Stay updated, free articles. Join our Telegram channel

Join