Rheumatic Fever Disease

DESCRIPTION

Rheumatic fever is an inflammatory disease, possibly autoimmune in nature. Rheumatic fever involves many tissues, including the heart, joints, skin, and central nervous system. Preceding infection of the upper respiratory tract with group A Streptococcus is a prerequisite to the development of acute rheumatic fever.

Rheumatic fever can cause permanent cardiac valvular disease as well as acute cardiac decompensation.
Recurrences are common if not prevented with "prophylactic" antibiotic treatment. In recent years there have been multiple reports of recurrences in adults as well as children.
System(s) affected: Cardiovascular, Hemic/Lymphatic/Immunologic, Nervous, Musculoskeletal, Skin/Exocrine
Genetics: A specific genetic marker that correlates with susceptibility to rheumatic fever has not been found, but the disease is known to occur in families
Incidence/Prevalence in USA:
- The incidence of rheumatic fever in the United States has been showing an overall decline for decades. In the 1970s it was a rare disease with an incidence of 0.5–1.88 cases per 100,000. However, since the mid-1980s there has been a resurgence of cases with multiple outbreaks reported in the U.S.
- The incidence calculated based on recent outbreaks has been as high as 18.1 per 100,000 in children aged 5–17 years.
Predominant age: Most common in children ages 5-15. Recurrences can be seen in adulthood.
Predominant sex: Male = Female

SIGNS AND SYMPTOMS

Joint symptoms ranging from arthralgias to frank arthritis (75%)
Joints involved are medium to large (e.g., ankles, knees, wrists)
Joint involvement is classically migratory
Joint symptoms usually disappear in 3–4 weeks without permanent deformities
Carditis (65%), mild or severe, often associated with murmurs
Cardiac involvement may include pericarditis, myocarditis, and/or valvular insufficiency; appears within 2 weeks and lasts 6 weeks to 6 months
Valvular damage may be permanent
P–R prolongation on ECG
Erythema marginatum (classic rash) < 5%
Subcutaneous nodules (painless, hard swellings overlying bony prominences) 5–10%
Chorea is often a late finding but may be a presenting complaint; occurs in 10–15% of patients and its duration is not altered by treatment
Fever 101–104°F (38.3–40.0°C)
Abdominal pain is common and may be severe
Epistaxis (historically important but rarely seen in acute rheumatic fever)
Facial tics
Facial grimace

CAUSES

Autoimmune mechanisms
A preceding upper respiratory infection with group A Streptococcus is a prerequisite

RISK FACTORS

Crowded living, school, or working conditions
Tendency to upper respiratory infections

LABORATORY

Increased acute phase reactants, including erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)
Bacteriological or serological evidence of group A streptococcal infection (e.g., antistreptolysin O [ASO], Streptozyme, or anti-deoxyribonuclease B [anti-DNase B])
Anemia

Drugs that may alter lab results: Prior treatment with aspirin or steroids
Disorders that may alter lab results: N/A

PATHOLOGICAL FINDINGS

Subcutaneous nodules have a characteristic histological appearance
Pericardial effusion
Fibrinous pericardium

SPECIAL TESTS

N/A

IMAGING

Chest X-ray
Echocardiogram (reveals pericardial effusion and documents valvular disease)

DIAGNOSTIC PROCEDURES

Throat cultures for Group A beta-hemolytic streptococci
Diagnosis is dependent on fulfilling the modified Jones criteria of two major manifestations or one major and two minor manifestations. In either case, there must be evidence of preceding group A streptococcal infection. The five major criteria are carditis, arthritis, chorea, erythema marginatum, subcutaneous nodules. The minor criteria include fever, arthralgia (cannot use if arthritis was used as a major criteria), previous rheumatic fever, acute phase labs, prolonged P-R interval on EKG.

APPROPRIATE HEALTH CARE

Outpatient management
Initial hospitalization may be helpful to diagnose and establish patient stability

GENERAL MEASURES

Mainstay of therapy is anti-inflammatory treatment
Patients with arthritis: therapy aimed at relief of pain
Patients with carditis: therapy to suppress inflammation
Patients with arrhythmias: treat with appropriate antiarrhythmic agents

SURGICAL MEASURES

N/A

ACTIVITY

Initial bedrest with activity increasing gradually as tolerated
Advance activity cautiously if there is evidence of carditis

DIET

Regular; low sodium initially if the patient has carditis

PATIENT EDUCATION

Information available from the American Heart Association

PREVENTION/AVOIDANCE

Patients will need to be on prophylactic penicillin throughout childhood and possibly indefinitely during adulthood. Monthly injections of 1.2 million units of benzathine penicillin intramuscularly is the preferred treatment.
Adults should be treated for a minimum of five years after an attack. Some treat adults indefinitely if there has been valvular disease. Oral penicillin V-K, 125 mg twice daily, is an alternative to monthly injections. In the event of penicillin allergy, sulfadiazine 500 mg daily for children weighing less than 30 kg or 1 g daily for all. Other alternatives may also be used.
If patients have valvular damage from acute rheumatic fever, they will require bacterial endocarditis prophylaxis for dental and other high-risk procedures.

POSSIBLE COMPLICATIONS

Subsequent attacks of acute rheumatic fever secondary to streptococcal reinfection
Carditis
Mitral stenosis
Congestive heart failure

EXPECTED COURSE AND PROGNOSIS

Sequelae limited to the heart and dependent of severity of carditis during an acute attack

ASSOCIATED CONDITIONS

N/A

AGE-RELATED FACTORS

Pediatric: More common in children
Geriatric: N/A
Others: N/A

PREGNANCY

Residual valvular disease may be exacerbated by pregnancy. Refer pregnant patient to cardiologist for assistance in management.

OTHER NOTES

N/A

ABBREVIATIONS

N/A

Clinical Investigations

ROLE OF HOMOEOPATHY

Holistic healing that treats the person, not just the disease. Experience gentle, lasting wellness with expert constitutional care.