Case summary

Rheumatic heart disease: Acute rheumatic fever

Pratap Sagar Tiwari

Introduction :ARF

• RF is an acute, immunologically mediated,multisystem inflammatory disease involvingheart, joints, CNS, skin and other tissues thatoccurs a few wks (2-4 Wks) after an episode ofgroup A β-hemolytic streptococcal pharyngitis.

• RHD is the cardiac manifestation of RF and is a/winflammation of the valves, myocardium, orpericardium.

• ARF usually affects children (MC betwn 5-15 yrs)

Pathophysiology

• The condition is triggered by an immune-mediated response to infection with specificstrains of group A streptococci, which haveantigens that may cross-react with cardiacmyosin and sarcolemmal membrane protein.

• Antibodies produced against the streptococcalantigens cause inflammation in theendocardium, myocardium and pericardium,as well as the joints and skin.

Histology

• Aschoff nodules are pathognomonic and occuronly in the heart.

• They are composed of multinucleated giant cellssurrounded by macrophages and T lymphocytes.

• Are not seen until the subacute or chronic phasesof rheumatic carditis.

• Anitschkow cells are enlarged macrophagesfound within granulomas (called Aschoff bodies).

The Jones Criteria

Major Minor

Migratory arthritis (predominantly involving the large joints)Carditis and valvulitis (eg, pancarditis)Sydenham choreaErythema marginatumSubcutaneous nodules

• Fever, Arthralgia• Elevated acute phase

reactants [(ESR), (CRP)]

• Prolonged PR interval

The probability of acute rheumatic fever is high in the setting of group A streptococcal infection followed by two major manifestations or one major and two minor manifestations .

Evidence of Streptococcal pharyngitis

• Positive throat culture for group A beta-hemolytic streptococci

• Positive rapid streptococcal antigen test

• Elevated or rising antistreptolysin O antibodytiter.

There are 3 circumstances in which a presumptive DX of ARF can be made without strict adherence to the above criteria :

• Chorea as the only manifestation.

• Indolent carditis .

• Recurrent rheumatic fever in patients with a history of rheumatic fever or rheumatic heart disease.

Pharyngitis: GAS infection vs Viral Infection

F s/o GAS infection F s/o Viral Infection

Patient 5 to 15 years of ageFever, HeadacheSudden onset of sore throatBeefy, swollen, red uvulaPain with swallowingNausea, vomiting, abdominal painTender, enlarged anterior cervical nodesScarlet fever rashSoft palate petechiae (doughnut lesions”)Tonsillopharyngealerythema,exudates

ConjunctivitisCoryzaCoughDiarrheaHoarseness

Adapted from Gerber MA, Baltimore RS, Eaton CB, et al. endorsed by the American Academy of Pediatrics. Circulation. 2009;119(11):1543.

Carditis :50-60%

• A 'pancarditis' involves the endocardium, myocardium andpericardium to varying degrees.

• May manifest as breathlessness (due to HF or p.effusion),palpitations or chest pain (usually due to pericarditis orpancarditis).

• Other: tachycardia, cardiac enlargement and new orchanged cardiac murmurs.

• A soft systolic murmur due to MR is very common. A MDM(the Carey Coombs murmur) is typically due to valvulitis,with nodules forming on the MV leaflets.

• AR occurs in about 50% but TV and PV are rarely involved.• Pericarditis may cause chest pain, a pericardial friction rub.

Arthritis :60-75%

• MC major manifestation and tends to occur earlywhen streptococcal antibody titres are high.

• An acute painful asymmetric and migratoryinflammation of the large joints typically affectsthe knees, ankles, elbows and wrists.

• The joints are involved in quick succession andare usually red, swollen & tender for btn a dayand 4 wks.

• The pain characteristically responds to aspirin; ifnot, the diagnosis is in doubt.

Skin lesions: <5 %

• Erythema marginatum :The lesions start as redmacules (blotches) that fade in the centre butremain red at the edges and occur mainly on thetrunk and proximal extremities but not the face.

• Subcutaneous nodules :They are small (0.5-2.0cm), firm and painless, non pruritic and are bestfelt over extensor surfaces of bone or tendons.They typically appear more than 3 weeks afterthe onset of other manifestations .

Erythema marginatum / Subcutanousnodules

http://www.hxbenefit.com/erythema-marginatum.htmhttp://www.doctortipster.com/1789-rheumatic-fever.html l

Sydenham's chorea 2-30 %

• This is a late neurological manifestation that appears atleast 3-8 months after the episode of ARF, when all theother signs may have disappeared.

• It occurs in up to 1/3rd of cases and is more common infemales.

• Emotional lability may be the first feature and is typicallyfollowed by purposeless involuntary choreiformmovements of the hands, feet or face. Speech may beexplosive and halting.

• Spontaneous recovery usually occurs within a few months.• Approximately one-quarter of affected patients will go on

to develop chronic rheumatic valve disease.

Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis)

AHA Recommendations for Duration of Secondary Prophylaxis

Category of Patient Duration of Prophylaxis

RF without carditis For 5 years after the last attack or 21 years of age (whichever is longer)

RF with carditis but no residual valvular disease

For 10 years after the last attack, or 21 years of age (whichever is longer)

RF with persistent valvulardisease, evident clinically or on echocardiography

For 10 years after the last attack, or 40 years of age (whichever is longer). Sometimes lifelong prophylaxis.

Chronic rheumatic heart disease

• Chronic VHD develops in at least half of those affected by rheumatic fever with carditis. Two-thirds of cases occur in women.

• The mitral valve is affected in more than 90% of cases; the aortic valve is the next most frequently affected, followed by the tricuspid and then the pulmonary valve.

• Isolated MS accounts for about 25% and an additional 40% have mixed MS/MR.

Poststreptococcal reactive arthritis

• The latent period between the antecedentstreptococcal infection -migratory arthritis isshorter (1-2wks) than the 2-3 wks usually seen inARF.

• The response of arthritis to aspirin is poor• Evidence of carditis is not seen, and the severity

of the arthritis is quite marked.• Extraarticular manifestations ie tenosynovitis &

renal abnormalities often are seen .• Acute phase reactants (ESR, CRP) tend to be

lower than of ARF.

End of slides

Ref:

• 1st slide pic :www.pathguy.com/lectures/heart.htm

• Harrison’s Principles of Internal medicine

• Davidson 21st ed

• Uptodate 20.3

• Medscape