RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

Rido Maulana
Rudini

Resource person : dr. Anna Ulfah Rahajoe, Sp.JP(K)

Outline
• Epidemiology
• Pathogenesis
• Clinical Features
• Diagnosis
• Treatment
• Prevention
– Rheumatic Fever initiated by a group A betahemolytic Streptococcal (GAS)
pharyngeal infection.
– Rheumatic Fever is the leading cause of acquired heart disease in children and
young adults.
– The illness is characterized by acute inflamation of:
• The heart
• Joints
• Skin
• Subcutaneous tissue
• Central nervous system

Mann DL, Zipes DP, Libby P, Bonow RO. Braunwalds Heart Disease. 10th Ed. Philadelphia ; Saunders: 2015. Pg. 1834-41.
Acute Rheumatic Fever
◦Acute rheumatic fever (ARF) results from the body’s autoimmune response to a
throat infection caused by Streptococcus pyogenes, also known as the group
A Streptococcus bacteria. 
◦Rheumatic heart disease (RHD) refers to the long-term cardiac damage caused by
either a single severe episode or multiple recurrent episodes of ARF.
◦The development of ARF occurs approximately two weeks after S. pyogenes
infection (Gewitz, et al., 2015).
 Epidemiology
• Decrease in incidence
• Improved socioeconomy
• Less overcrowded housing
• Improved access to medical care
• Resurgence
• Post-Soviet weakening of health
care system & economic crisis

Mayosi MB. Rheumatic fever. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E. Braunwald’s heart disease: a textbook of cardiovascular medicine.
11th ed. Philadelphia: Elsevier; 2019. p.1510-1517.
Pathogenesis

Sheel M., Moreland N. J., Fraser J. D.,

Carapetis J. Development of Group A
streptococcal vaccines: an unmet global health
need. Expert Review of Vaccines.
2016;15(2):227–238. PubMed PMID:
Sheel M., Moreland N. J., Fraser J. D.,
Carapetis J. Development of Group A
streptococcal vaccines: an unmet global
health need. Expert Review of Vaccines.
2016;15(2):227–238. PubMed PMID:
26559880.
Revised Jones Criteria for ARF
Revised Jones Criteria for ARF
 Clinical Features: Carditis
• Carditis = inflammation affects
all heart layers (pericardium,
myocardium, endocardium)
• 40-91% of patients
• Autoimmune cross-reactivity
between bacterial & cardiac
antigens
• Aschoff bodies = focal fibrinoid
necrosis surrounded by
inflammatory cells 🡪 fibrous scar
tissue
Ryznar E, O’Gara PT, Lilly LS. Valvular heart disease. In: Lilly LS. Pathophysiology of heart disease: a collaborative project of medical students and faculty. 6 th ed. Philadelphia: Wolters Kluwer;
2016. p.192-220.
Mayosi MB. Rheumatic fever. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E. Braunwald’s heart disease: a textbook of cardiovascular medicine. 11 th ed. Philadelphia:
Elsevier; 2019. p.1510-1517.
 Clinical Features: Carditis
• Most common: MR (pansystolic murmur) 🡪 endocarditis
• Tachycardia
• Impaired ventricular contractility 🡪 CHF 🡪 myocarditis
• Pericardial friction rub 🡪 pericarditis
• Transient heart murmurs (turbulent flow across inflamed valve
leaflets)
• Heart blocks

Ryznar E, O’Gara PT, Lilly LS. Valvular heart disease. In: Lilly LS. Pathophysiology of heart disease: a collaborative project of medical students and faculty. 6 th ed.
Philadelphia: Wolters Kluwer; 2016. p.192-220.
Mayosi MB. Rheumatic fever. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E. Braunwald’s heart disease: a textbook of cardiovascular medicine.
11th ed. Philadelphia: Elsevier; 2019. p.1510-1517.
 Clinical Features: Arthritis
• Most common
• Migratory / additive (untreated:
6-16 joints)
• Asymmetric
• Lower limbs 🡪 upper limbs
• Large joints > small joints
• Monoarthritis: 17-25%
• Usually <4 weeks 🡪 consider
other conditions
Mayosi MB. Rheumatic fever. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E. Braunwald’s heart disease: a textbook of cardiovascular medicine.
11th ed. Philadelphia: Elsevier; 2019. p.1510-1517.
 Clinical Features: Sydenham Chorea
• Involuntary, purposeless, jerky movements associated with hypotonia
& weakness
• More common in females
• Longer latent period (6-8 weeks), lasts 8-15 weeks
• Interfere with voluntary activity, but disappear during sleep
• Jack-in-the-box tongue, milking sign
• Emotional lability

Mayosi MB. Rheumatic fever. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E. Braunwald’s heart disease: a textbook of cardiovascular medicine.
11th ed. Philadelphia: Elsevier; 2019. p.1510-1517.
 Clinical Features: Subcutaneous Nodules
• Elbows, knees, ankles, Achilles
tendons
• Firm, painless, freely movable
• 0.5-2 cm
• Usually in children with
prolonged active carditis
• <1 month

Mayosi MB. Rheumatic fever. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E. Braunwald’s heart disease: a textbook of cardiovascular medicine.
11th ed. Philadelphia: Elsevier; 2019. p.1510-1517.
 Clinical Features: Erythema Marginatum
• Less common
• Evanescent, pink, nonpruritic
• Central healing
• Irregular serpiginous border
• May become more prominent
after a hot shower

Mayosi MB. Rheumatic fever. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E. Braunwald’s heart disease: a textbook of cardiovascular medicine.
11th ed. Philadelphia: Elsevier; 2019. p.1510-1517.
 Treatment
• Bed rest: until fever subsided & CRP returns to normal
• Benzathine benzylpenicillin IM
• Anti-inflammatory agent: 1-3 months
• Aspirin 100 mg/kg/day in four or five doses
• Prednisone 1-2 mg/kg/day
• Surgical management

Mayosi MB. Rheumatic fever. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E. Braunwald’s heart disease: a textbook of cardiovascular medicine.
11th ed. Philadelphia: Elsevier; 2019. p.1510-1517.
 Primordial Prevention
• Socioeconomic status
• Increasing access to primary health care

Mayosi MB. Rheumatic fever. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E. Braunwald’s heart disease: a textbook of cardiovascular medicine.
11th ed. Philadelphia: Elsevier; 2019. p.1510-1517.
Management
 Primary Prevention

Mayosi MB. Rheumatic fever. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E. Braunwald’s heart disease: a textbook of cardiovascular medicine.
11th ed. Philadelphia: Elsevier; 2019. p.1510-1517.
Secondary Prevention

Mayosi MB. Rheumatic fever. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald
E. Braunwald’s heart disease: a textbook of cardiovascular medicine. 11th ed. Philadelphia:
Elsevier; 2019. p.1510-1517.
 Conclusion
• Rheumatic Fever is an autoimmune processes from the infection of
GAS.
• It is a multifactorial disease that will happen if there are the host, the
agent and the environment.
• It can affect heart, joints, skin, brain and subcutaneous tissue.
• Carditis is the most serious problem in RF
• Early diagnosis and appropriate treatment is important to get the
good result.
THANK YOU