Description

Guidelines for the initial attack of rheumatic fever were initially formulated by Jones in 1944, with periodic updates by the American Heart Association. No single symptom, sign or laboratory test is pathognomonic for acute rheumatic fever.


Evidence of antecedent group A streptococcal infection may include:

(1) positive throat culture for group A streptococcus (Streptococcus pyogenes)

(2) positive rapid group A streptococcal antigen test

(3) elevated or rising streptococcal antibody titer (normal ASO: preschool <= 85 Todd units; school age <= 170 Todd units, adult <= 85 Todd units; from Wallach 1996)

 

Major manifestations:

(1) carditis

(1a) valvulitis: rheumatic carditis should be suspected in a patient who does not have a history of rheumatic heart disease who develops a new apical systolic murmur of mitral regurgitation (with or without an apical mid-diastolic murmur) and/or a basal diastolic murmur of aortic regurgitation

(1b) isolated myocarditis or pericarditis should be labeled as rheumatic in origin with caution in the absence of murmur suggesting valvulitis

(1c) myocarditis: tachycardia, cardiomegaly, congestive heart failure

(1d) pericarditis: distant heart sounds, friction rub, and chest pain

(2) polyarthritis

(2a) most frequently involves knees, ankles, elbows and wrists

(2b) involvement limited to small joints of hand or feet suggests another diagnosis

(2c) symptoms include swelling, heat, redness, severe pain, tenderness to touch and limitation of motion

(2d) salicylates result in dramatic improvement within a few hours of administration

(3) chorea

(3a) purposeless, involuntary, rapid movements of the trunk and/or the extremities often associated with muscle weakness and emotional lability

(4) erythema marginatum

(4a) the erythematous areas may have pale centers with rounded or serpiginous margins

(4b) not pruritic or indurated

(4c) erythema blanches on pressure

(4d) may be induced by the application of heat

(4e) migratory and transient

(5) subcutaneous nodules

(5a) firm, painless nodules over the extensor surfaces of larger joints, the occipital region or over the spinous processes of the thoracic or lumber vertebrae

(5b) overlying skin moves freely and is not inflamed

 

Minor Manifestations

Finding

clinical findings

arthralgias (do not count as minor manifestation if arthritis is present)

 

fever >= 39°C (may be absent if treated with antipyretic agents)

laboratory findings

elevated acute phase reactants

• erythrocyte sedimentation rate

• C-reactive protein

ECG findings

prolonged PR interval

 

where:

• The normal C reactive protein concentration is < 1 mg/dL.

• The upper limit of normal for erythrocyte sedimentation rate (ESR)

 

Age

Male

Female

child

10 mm/h

10 mm/h

adult <= 50

15 mm/h

20 mm/h

adult > 50

20 mm/h

30 mm/h

 

 

• The upper limit of normal for P-R interval and heart rate (Krupp, 1979)

 

Age

Rate < 70

Rate 71-90

Rate 91-110

Rate 111-130

Rate > 130

0-1.5

0.16

0.15

0.145

0.135

0.125

1.5-6

0.17

0.165

0.155

0.145

0.135

7-13

0.18

0.17

0.16

0.15

0.14

14-17

0.19

0.18

0.17

0.16

0.15

adult (large)

0.21

0.20

0.19

0.18

0.17

 

 

 

High probability of acute rheumatic fever - both of the following:

(1) evidence of antecedent group A streptococcal infection

(2) one or both of the following:

(2a) 2 major manifestations

(2b) 1 major and 2 minor manifestations

 

Exceptions to Jones criteria:

(1) Chorea may be the only manifestation of rheumatic fever.

(2) Indolent carditis may be the only manifestation in patients who seek medical attention months after the initial onset of rheumatic fever.

(3) Recurrent attacks of rheumatic fever can occur and need to be distinguished from relapse of the previous episode.

(4) Diagnosis in these situations are considered presumptive until other causes have been excluded.


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