Can rheumatic heart disease present without fever (afebrile) in a patient with a history of rheumatic fever?

Can Rheumatic Heart Disease Present Without Fever?

Yes, rheumatic heart disease (RHD) and even acute rheumatic fever (ARF) can present without fever (afebrile), particularly in certain populations and clinical scenarios. 1

Fever as a Minor Criterion: Not Always Present

While fever is traditionally considered a minor manifestation of acute rheumatic fever, the absence of fever does not exclude the diagnosis, and fever thresholds vary significantly by population risk level. 1

Temperature Thresholds Vary by Population Risk

• In high-risk populations (such as Aboriginal Australians), fever defined as >38°C was present in only 75% of ARF cases, and only 25% had fever >39°C 1
• Critically, 41% of individuals in high-risk populations who were not diagnosed with ARF due to absence of fever (when defined as ≥38°C or ≥39°C) subsequently developed ARF or RHD 1
• A cutoff of >37.5°C would have captured 90% of suspected ARF cases in high-risk populations 1
• In low-risk populations, fever associated with ARF usually exceeds 38.5°C orally, but this is not universal 1

Important Clinical Context

• The widespread availability of antipyretic agents means patients may appear afebrile despite having had fever, requiring a detailed history to understand the true clinical picture 1
• Fever is classified as a minor manifestation in the Jones criteria, not a major one, meaning ARF can be diagnosed without it if major criteria and other supporting evidence are present 1

Specific Afebrile Presentations

Isolated Chorea (Sydenham Chorea)

Patients with isolated chorea represent a unique subset where inflammatory markers and fever are characteristically absent. 1

• Normal erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels are typical in isolated chorea, unlike other ARF presentations 1
• This occurs due to the long latent period between the streptococcal infection and onset of chorea, during which the acute inflammatory phase has resolved 1
• Evidence of recent group A streptococcal infection may be difficult or impossible to document in these cases 1

Chronic Rheumatic Heart Disease

Established RHD (the chronic valvular sequelae following ARF) is not an acute inflammatory condition and does not present with fever unless there is: 1, 2

• A recurrent episode of acute rheumatic fever
• Complications such as infective endocarditis
• Heart failure (which itself does not cause fever)

60-65% of patients who recover from initial ARF episodes develop chronic valvular heart disease, which manifests as progressive valve damage, atrial fibrillation, and heart failure—none of which inherently cause fever. 1

Critical Diagnostic Pitfalls

Do Not Rely on Fever Alone

• Normal ESR and CRP should prompt serious reconsideration of ARF diagnosis, except in patients with isolated chorea 1
• In typical ARF (excluding isolated chorea), CRP values should always be elevated above the upper limit of normal and are commonly >7.0 mg/dL 1
• ESR is typically >60 mm in the first hour, though some experts consider >30 mm/h consistent with ARF 1

Subclinical Presentations

• At least one-third of rheumatic fever cases arise from asymptomatic group A streptococcal infections, meaning patients may never have had recognized pharyngitis or fever 3, 4
• Echocardiographic screening has identified subclinical RHD (silent valve lesions) at rates several-fold higher than clinical estimates, suggesting many cases progress without obvious acute febrile illness 1, 2

Clinical Algorithm for Afebrile Suspected RHD

When evaluating a patient with suspected rheumatic disease who is afebrile:

1. Determine population risk level (high-risk: endemic areas, indigenous populations, low socioeconomic status; low-risk: developed countries with low ARF incidence) 1

2. Assess for major Jones criteria:

   • Carditis (clinical or echocardiographic evidence of valvulitis)
   • Polyarthritis or monoarthritis/polyarthralgia (depending on risk population)
   • Chorea
   • Erythema marginatum
   • Subcutaneous nodules 1

3. Check inflammatory markers:

   • If ESR and CRP are normal AND patient does not have isolated chorea, strongly reconsider the diagnosis 1
   • If ESR and CRP are elevated, absence of fever does not exclude ARF 1

4. Obtain detailed history:

   • Recent antipyretic use (may mask fever) 1
   • Symptoms of pharyngitis in preceding weeks
   • Previous episodes of ARF or family history of RHD 3, 5

5. Document evidence of preceding streptococcal infection (though this may be impossible in chorea due to long latent period) 1

Management Implications

Even in afebrile presentations, if ARF or RHD is diagnosed, the treatment approach remains unchanged: 1, 3, 4

• Full 10-day course of penicillin to eradicate residual group A streptococcus 3, 5
• Immediate initiation of long-term secondary prophylaxis with benzathine penicillin G 1.2 million units IM every 4 weeks (or every 3 weeks in high-risk situations) 1, 3, 4
• Duration of prophylaxis depends on cardiac involvement: ≥10 years or until age 40 (whichever is longer) for those with persistent valvular disease 1, 3, 4

The absence of fever should never delay appropriate antibiotic prophylaxis in patients with confirmed or suspected RHD, as recurrent episodes cause progressive cardiac damage. 1, 4