Rheumatic Fever Clinical Presentation
- Author: Mark R Wallace, MD, FACP, FIDSA; Chief Editor: Michael Stuart Bronze, MD more...
Rheumatic fever manifests as various signs and symptoms that may occur alone or in various combinations.
Although estimates vary, only 35%-60% of patients with rheumatic fever recall having any upper respiratory symptoms in the preceding several weeks. Many symptomatic individuals do not seek medical attention, go undiagnosed, or do not take the prescribed antibiotic for acute rheumatic fever (ARF) prevention.[6, 11] If a course of penicillin or another appropriate antibiotic is taken at this time, the risk of ARF is reduced by approximately 80%.
Overall, arthritis occurs in approximately 75% of first attacks of ARF. The likelihood increases with the age of the patient, and arthritis is a major manifestation of ARF in 92% of adults.
The arthritis of ARF is usually symmetrical and involves large joints, such as the knees, ankles, elbows, and wrists. Tenosynovitis is common in adults and may be severe enough to suggest a diagnosis of disseminated gonococcal disease.
The evolution of arthritis in individual joints tends to overlap; therefore, multiple joints may be inflamed simultaneously, causing more of an additive than a migratory pattern.
Monoarticular arthritis can occur in ARF if NSAIDs are used early in the course. Although not recognized in the Jones criteria, this is included as a major manifestation in the New Zealand diagnostic criteria for ARF.
In most instances, the entire bout of polyarthritis subsides within 4 weeks without any permanent damage. If not, a different diagnosis should be entertained.
Of first attacks of ARF, carditis occurs in 30%-60% of cases. It is more common in younger children but does occur in adults.
Severe inflammation can cause congestive heart failure (CHF).
Patients with carditis may present with shortness of breath, dyspnea upon exertion, cough, paroxysmal nocturnal dyspnea, chest pain, and/or orthopnea. Carditis may also be asymptomatic and may be diagnosed solely by auscultation or, perhaps, echocardiography (controversial; see Physical).
This occurs in up to 25% of ARF cases in children but is very rare in adults. It is more common in girls. Sydenham chorea in ARF is likely due to molecular mimicry, with autoantibodies reacting with brain ganglioside.
Sydenham chorea may occur with other symptoms or as an isolated finding. It typically presents 1-6 months after the precipitating streptococcal infection and usually has both neurologic and psychological features.
In the isolated form, laboratory evidence of a preceding streptococcal infection may be lacking.
Like the polyarthritis, Sydenham chorea usually resolves without permanent damage but occasionally lasts 2-3 years and be a major problem for the patient and her family.
In first attacks of ARF in children, erythema marginatum occurs in approximately 10%. Like chorea, it is very rare in adults.
Patients or parents may report a nonpruritic, painless, serpiginous, erythematous eruption on the trunk. It is usually noted only in fair–skinned patients.
The lesions may persist intermittently for weeks to months.
Subcutaneous nodules are rarely noticed by the patient (see Physical).
Other symptoms may include fever, abdominal pain, arthralgia, malaise, and epistaxis.
Joint involvement in ARF may range from arthralgia to frank polyarthritis characterized by swelling, redness, warmth, and joint tenderness. Monoarticular arthritis may occur if anti-inflammatory agents are used early in the course.
The joints frequently involved include the knees, ankles, elbows, and wrists. The small joints of the hands and the spine are rarely involved. Hand involvement tends to occur in poststreptococcal arthritis, a controversial related syndrome without a risk of carditis.
Inflammation begins to subside within a few days to a week and disappears within 2-4 weeks.
The arthritis is classically described as migratory, but, in many cases, new joints are affected before the previously involved joints improve, leaving the appearance of an additive arthritis.
In most cases, the process does not leave any residual damage. On very rare occasions, periarticular fibrosis occurs after rheumatic arthritis, the so-called Jaccoud joint.
Carditis is the only manifestation of ARF with significant potential to cause long-term disability and/or death. It is usually a pancarditis involving the pericardium, myocardium, and endocardium.
The signs of carditis include the development of new murmurs, cardiac enlargement, CHF, pericardial friction rub, and/or pericardial effusion.
Characteristic murmurs of acute carditis include the high-pitched, blowing, holosystolic, apical murmur of mitral regurgitation; the low-pitched, apical, mid-diastolic, flow murmur (Carey-Coombs murmur); and a high-pitched, decrescendo, diastolic murmur of aortic regurgitation heard at the aortic area. Murmurs of mitral and aortic stenosis are observed in chronic valvular heart disease. Isolated aortic disease is distinctly unusual.
The features of CHF include tachycardia, a third heart sound, rales, and edema.
Pericarditis presents as a pericardial rub or effusion.
The use of echocardiography to detect carditis often reveals "subclinical" rheumatic cardiac disease (both acute and chronic) not appreciated by the standard examination.[19, 20, 21] Whether these subclinical carditis findings carry the same clinical importance as those detected by standard auscultation is unclear; echocardiography findings alone do not currently count toward fulfillment of the Jones criteria used in most countries, but are included in the diagnostic criteria used in New Zealand and Australia. It is unclear whether such subclinical carditis necessitates secondary antibiotic prophylaxis (see Diagnosis). This issue is the most contentious in the rheumatic fever literature[23, 24] and has major implications for diagnosis and screening. For now, clinicians must rely on their best judgment when evaluating possible carditis detected only with echocardiography.
Subcutaneous nodules are uncommon and are usually associated with severe carditis. They tend to occur several weeks after illness onset, are usually painless, and usually go unnoticed by the patient.
They are found primarily over the bony surfaces or prominences and in tendon sheaths. The common sites include the elbows, knees, wrists, ankles, over the Achilles tendon, the back of the scalp, and spinous process of the vertebrae.
The individual lesions of erythema marginatum are evanescent, moving over the skin in serpiginous patterns. Likened to smoke rings, they have a tendency to advance at the margins while clearing in the center.
The lesions may be macular and can develop and disappear in minutes, appearing to change shape while being examined.
They are found on the trunk and proximal aspects of the extremities and often go unnoticed by patients and parents, as they are usually covered by clothing.
This is a neurological disorder characterized by emotional lability, personality change, muscular weakness, and uncoordinated, involuntary, purposeless movements.
The classic weakness is characterized by the inability to sustain a tetanic contraction. Patients are unable to maintain a clenched fist when attempting to grip the examiner's hand. Other findings include dysarthric speech, gait problems, and poor fine-motor skills.
The motor symptoms usually disappear during sleep and may be partially suppressed by sedation.
They can involve the face, hands, and feet.
Untreated rheumatic fever/chronic rheumatic fever
The average duration of an untreated ARF attack is 3 months. Chronic rheumatic fever, generally defined as disease persisting for longer than 6 months, occurs in less than 5% of cases.
Group A beta-hemolytic streptococcal infection may lead to rheumatic fever. The overall attack rate after streptococcal pharyngitis 0.3-3%, but certain genetically predisposed individuals, comprising perhaps 3%-6% of the population, account for those who develop rheumatic fever.
Studies in developed countries have established that rheumatic fever followed only pharyngeal infections and that not all serotypes of group A streptococci cause rheumatic fever. For example, some strains (eg, M types 4, 2, 12) in a population susceptible to rheumatic disease do not result in recurrences of rheumatic fever. The classic rheumatogenic serotypes are thought to include 3, 5, 6, 14, 18, 19, and 24. More recent data, largely from studies of the indigenous peoples of Australia, suggest that skin infections (pyoderma) can predispose to ARF and that various other serotypes may be involved.[6, 5]
Two basic theories have been postulated to explain the development of ARF and its sequelae following group A streptococcal infection: (1) a toxic effect produced by an extracellular toxin of group A streptococci on target organs such as the myocardium, valves, synovium, and brain and (2) an abnormal immune response to streptococcal components. Increasing and compelling evidence now strongly favors the autoimmune explanation. It seems clear that an exaggerated immune response in a susceptible individual leads to rheumatic fever. This probably occurs through molecular mimicry, in which the immune response fails to differentiate between epitopes of the streptococcal pathogen and certain host tissues.[6, 8]
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