Lesson topic №29 Острая ревматическая лихорадка (Rheumatic fever)

Symptoms and Signs of Rheumatic Fever

•Joints

•Migratory polyarthritis is the most common manifestation of acute rheumatic fever, occurring in about 35 to 66% of children; it is often accompanied by fever.

•"Migratory" means the arthritis appears in one or a few joints, resolves but then appears in others, thus seeming to move from one joint to another.

•Occasionally monarthritis occurs in high-risk indigenous populations (eg, in Australia, India, Fiji) but very rarely in the US.

•Joints become extremely painful and tender; these symptoms are often out of proportion to the modest warmth and swelling present on examination (this is in contrast to the arthritis of Lyme disease, in which the examination findings tend to be more severe than the symptoms).

•Ankles, knees, elbows, and wrists are usually involved.

•Shoulders, hips, and small joints of the hands and feet also may be involved, but almost never alone. If vertebral joints are affected, another disorder should be suspected.

•Arthralgia-like symptoms may be due to nonspecific myalgia or tenodynia in the periarticular zone; tenosynovitis may develop at the site of muscle insertions.

•Joint pain and fever usually subside within 2 weeks and seldom last > 1 month.

Heart

Carditis can occur alone or in combination with pericardial rub, murmurs, cardiac enlargement, or heart failure.

In the first episode of acute rheumatic fever, carditis occurs in about 50 to 70%.

Patients may have high fever, chest pain, or both; tachycardia is common, especially during sleep.

In about 50% of cases, cardiac damage (ie, persistent valve dysfunction) occurs much later.

Although the carditis of ARF is considered to be a pancarditis (involving the endocardium, myocardium, and pericardium), valvulitis is the most consistent feature of ARF, and if it is not present, the diagnosis should be reconsidered.

The diagnosis of valvulitis has classically been made by auscultation of murmurs, but subclinical cases (ie, valvular dysfunction not manifested by murmurs but recognized on echocardiography and Doppler studies) may occur in up to 18% of cases of ARF.

Heart murmurs are common and, although usually evident early, may not be heard at initial examination; in such cases, repeated clinical examinations as well as echocardiography are recommended to determine the presence of carditis.

Mitral regurgitation is characterized by an apical pansystolic blowing murmur radiating to the axilla.

The soft diastolic blow at the left sternal border of aortic regurgitation, and the presystolic murmur of mitral stenosis, may be difficult to detect.

Murmurs often persist indefinitely. If no worsening occurs during the next 2 to 3 weeks, new manifestations of carditis seldom follow.

ARF typically does not cause chronic, smoldering carditis.

Scars left by acute valvular damage may contract and change, and secondary hemodynamic difficulties may develop in the myocardium without persistence of acute inflammation.

Pericarditis may be manifested by chest pain and a pericardial rub.

Heart failure caused by the combination of carditis and valvular dysfunction may cause dyspnea without rales, nausea and vomiting, a right upper quadrant or epigastric ache, and a hacking, nonproductive cough. Marked lethargy and fatigue may be early manifestations of heart failure.

Skin

Cutaneous and subcutaneous features are uncommon and almost never occur alone, usually developing in a patient who already has carditis, arthritis, or chorea.

Subcutaneous nodules, which occur most frequently on the extensor surfaces of large joints (eg, knees, elbows, wrists), usually coexist with arthritis and carditis. Fewer than 10% of children with acute rheumatic fever have nodules. Ordinarily, the nodules are painless and transitory and respond to treatment of joint or heart inflammation.

Erythema marginatum is a serpiginous, flat or slightly raised, nonscarring, and painless rash. Fewer than 6% of children have this rash. The rash usually appears on the trunk and proximal extremities but not the face. It sometimes lasts < 1 day. Its appearance is often delayed after the inciting streptococcal infection; it may appear with or after the other manifestations of rheumatic inflammation.

Erythema Marginatum in Rheumatic Fever

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Erythema marginatum is a serpiginous rash seen in patients with rheumatic fever.

CNS

Sydenham chorea occurs in about 10 to 30% of children.

It may develop along with other manifestations but frequently arises after the other manifestations have subsided (often months after the acute streptococcal infection) and thus may be overlooked as an indicator of

acute rheumatic fever.

Onset of chorea is typically insidious and may be preceded by inappropriate laughing or crying.

Chorea consists of rapid and irregular jerking movements that may begin in the hands but often becomes generalized, involving the feet and face.

Characteristic findings include fluctuating grip strength (milkmaid’s grip), tongue fasciculations or tongue darting

(the tongue cannot protrude without darting in and out), facial grimacing, and explosive speech with or without

tongue clucking.

Associated motor symptoms include loss of fine motor control, and weakness and hypotonia (that can be severe enough to be mistaken for paralysis).

Previously undiagnosed obsessive-compulsive behavior may be unmasked in many patients.

Other

Fever (≥ 38.5° C) and other systemic manifestations such as anorexia and malaise can be prominent but are not specific. ARF can occasionally manifest as fever of unknown origin until a more identifiable sign develops.

Abdominal pain and anorexia can occur because of the hepatic involvement in heart failure or because of concomitant mesenteric adenitis, and rarely the situation may resemble acute appendicitis.

Recurrence

Recurrent episodes of ARF often mimic the initial episode;

carditis tends to recur in patients who have had moderate to severe carditis in the past, and chorea without carditis recurs in patients who had chorea without carditis initially.

Diagnosis of Rheumatic Fever

Modified Jones criteria (for initial diagnosis)

Testing for GAS (culture, rapid strep test, or antistreptolysin O and anti-DNase B titers)

ECG

Echocardiography with Doppler

Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level

Diagnosis of a first episode of acute rheumatic fever (ARF) is based on the modified Jones criteria (1; see Table:

Modified Jones Criteria for a First Episode of Acute Rheumatic Fever (ARF)*);

2 major criteria or 1 major and 2 minor criteria are required, each along with evidence of preceding group A streptococcal (GAS) infection.

Sydenham chorea alone (ie, without minor criteria) fulfills diagnostic criteria if other causes of movement disorder are ruled out.

The modified Jones criteria were designed for the evaluation of acute rheumatic fever rather than for a possible recurrence.

However, if patients have a reliable past history of acute rheumatic fever or rheumatic heart disease and also have documented group A streptococcal infection, the criteria may be used to establish the presence of a recurrence.

A preceding streptococcal infection is suggested by a recent history of pharyngitis and is confirmed by one or more of the following:

Positive throat culture

Increased or preferably rising antistreptolysin O titer

Positive rapid GAS antigen test in a child with clinical manifestations suggestive of streptococcal pharyngitis

Recent scarlet fever is highly suggestive. Throat cultures and rapid strep antigen tests are often negative by the time ARF manifests, whereas titers of antistreptolysin O and anti-DNase B typically peak 3 to 6 weeks after GAS pharyngitis.

About 80% of children with ARF have a significantly elevated antistreptolysin O titer;

if an anti-DNase B antibody level is also done, the percentage with confirmed GAS infection is higher, especially if acute and convalescent samples are tested.