DIAGNOSIS OF ACUTE RHEUMATIC FEVER
Gusti Ayu Riska Pertiwi1002005069
Faculty of Medicine Udayana University
INTRODUCTION
• Acute Rheumatic fever (ARF) is a nonsuppurative, immune-mediated inflammatory disease, which occurs as a delay sequel to group A, β-hemolytic streptococcus (GABHS) pharyngitis 1-4.
• Affects connective tissue of the heart, joints, skin and vessels1-4.
• Rheumatic Heart Disease (RHD) as squale condition of ARF can leading to congestive heart failure, strokes, endocarditis, and death 1-4
INTRODUCTION
1Seckeler MD and Hoke TR. The worldwide epidemiology of acute rheumatic fever and rheumatic heart disease Clinical Epidemiology 2011;3:67–84.1
2 National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia, An evidence-based review. 2006; Pp. 7-26.3Kaplan EL. Rheumatic Fever. In: Fauci AS (eds). Harrison’s rheumatology. New York: McGraw-hill; 2006. Pp 105-108.4WHO Expert Consultation on Rheumatic Fever and Rheumatic Heart Disease. Rheumatic fever and rheumatic heart disease: report of a WHO Expert Consultation, Geneva, 29 Oct – 1 Nov 2001. Geneva: WHO 2004. (WHO Technical Report Series No. 923).
O GABHS “rheumatogenic” O Susceptible individual O Environmental risks
INTRODUCTION cont.
How does ARF develop?
small proportions of people in any population (3-5%) have an inherent
susceptibility to ARF 6,7
6Lawrenson J. Rheumatic fever: New ideas in diagnosis and management. SAHeart 2010;7:252-257.7Beggs S, Peterson G, Tompson A. Antibiotic use for the prevention and treatment of rheumatic fever and rheumatic heart disease in children. Report for the 2nd Meeting of World Health Organization’s subcommittee of the Expert Committee of the Selection and Use of Essential Medicines. Geneva: 2008. Pp. 3
An estimated 12 million people are affected by ARF and RHD globally4,9
An estimated 12 million people are affected by ARF and RHD globally4,9
Up to 150 cases per 100 000 population in developing countries versus 1 case per 100 000 population in developed countries such as the United States4,9
Up to 150 cases per 100 000 population in developing countries versus 1 case per 100 000 population in developed countries such as the United States4,9
Predominantly affects children aged 5–14 years, rare affect children less than 3 years old or adults2,7
Predominantly affects children aged 5–14 years, rare affect children less than 3 years old or adults2,7
INTRODUCTION cont..
2 National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia, An evidence-based review. 2006; pg7-26.4WHO Expert Consultation on Rheumatic Fever and Rheumatic Heart Disease. Rheumatic fever and rheumatic heart disease: report of a WHO Expert Consultation, Geneva, 29 Oct – 1 Nov 2001. Geneva: WHO 2004. (WHO Technical Report Series No. 923).7Beggs S, Peterson G, Tompson A. Antibiotic use for the prevention and treatment of rheumatic fever and rheumatic heart disease in children. Report for the 2nd Meeting of World Health Organization’s subcommittee of the Expert Committee of the Selection and Use of Essential Medicines. Geneva: 2008. Pp. 39Miyake CY, Gauvreau K, Tani LY, Sundel RP. Newburger JW. Characteristics of Children Discharged From Hospitals in the United States in 2000 With the Diagnosis of Acute Rheumatic Fever. Pediatrics 2007;120:503-508.
Recurrenct may occurs well into their fourties 40 y.o2,7Recurrenct may occurs well into their fourties 40 y.o2,7
CONTENT
two major criteria OR one major criterion and two minor criterion,
PLUSevidence of antecedent streptococcal infection1-
5,11
2.1 Diagnosis Based on Jones’s Criteria Jones’s criteria consist major and
minor criteria
To fulfill Jones criteria:
CONTENT
Tabel 1. The Jones Criteria for Acute Rheumatic Fever, Update 19923
Major Criteria Minor Criteria
Carditis Clinical
Fever
Polyarthritis Arthralgia
ChoreaLaboratory
Acute-phase reactants—
erythrocyte sedimentation rate,
C-reactive protein
Erythema marginatum Electrocardiogram—prolonged
PR intervalSubcutaneous nodules
Plus supporting evidence of antecendent GABHS infection :
Increased antistreptolysin O (ASO) or other streptococcal
antibodies (DNAse B)
Positive throat culture for GABHS
Positive rapid antigen detection test for GABHS
3Kaplan EL. Rheumatic Fever. In: Fauci AS (eds). Harrison’s rheumatology. New York: McGraw-hill; 2006. Pp 105-108.
Tabel 2. 2002–2003 WHO criteria for the diagnosis of rheumatic fever and rheumatic heart disease (based on the revised Jones criteria) 2,4,11
2National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia, An evidence-based review. 2006; Pp. 7-26.4WHO Expert Consultation on Rheumatic Fever and Rheumatic Heart Disease. Rheumatic fever and rheumatic heart disease: report of a WHO Expert Consultation, Geneva, 29 Oct – 1 Nov 2001. Geneva: WHO 2004. (WHO Technical Report Series No. 923).11Cilliers AM. Clinical review: Rheumatic fever and its management. BMJ 2006;333:1153–1156.
• 40% to 60% of patients with ARF have evidence of carditis
• Carditis is typically valvulitis, and has been traditionaly diagnosed by a murmur suggestive of valvar regurgitation
• There may be pericarditis and myocarditis
• Echocardiography as supportive examination
Jones’s Major Criteria
Carditis
Clinical diagnosis of carditis is based on3,4:the presence of significant murmurs
apical systolic murmur of mitral regurgitation
and/or the basal diastolic murmur of aortic regurgitation
tachycardia, pericardial friction rub cardiomegaly
Carditis cont.
Jones’s Major Criteria
3Kaplan EL. Rheumatic Fever. In: Fauci AS (eds). Harrison’s rheumatology. New York: McGraw-hill; 2006. Pp 105-108.4WHO Expert Consultation on Rheumatic Fever and Rheumatic Heart Disease. Rheumatic fever and rheumatic heart disease: report of a WHO Expert Consultation, Geneva, 29 Oct – 1 Nov 2001. Geneva: WHO 2004. (WHO Technical Report Series No. 923).
Asymmetrical redness, swelling, and intense pain of multiple joints, that can be migratory or additive 1-3
Affect large joint (ankles, knee, wrists, and elbow, seldom involve the hip joints); not the small joint3,4
It occurs at early course of the disease3,4
Shoud be differentiated from PSRA11
Jones’s Major Criteria
Polyarthritis
1Seckeler MD and Hoke TR. The worldwide epidemiology of acute rheumatic fever and rheumatic heart disease Clinical Epidemiology 2011;3:67–84.3Kaplan EL. Rheumatic Fever. In: Fauci AS (eds). Harrison’s rheumatology. New York: McGraw-hill; 2006. Pp 105-108.4WHO Expert Consultation on Rheumatic Fever and Rheumatic Heart Disease. Rheumatic fever and rheumatic heart disease: report of a WHO Expert Consultation, Geneva, 29 Oct – 1 Nov 2001. Geneva: WHO 2004. (WHO Technical Report Series No. 923).11Cilliers AM. Clinical review: Rheumatic fever and its management. BMJ 2006;333:1153–1156.
rapid, uncontrolled movements, especially affecting the hands, feet, tongue and face2-4
Bilateral or unilateral (hemichorea) 2-4 Latency period 1-7 months2-4
Females > male, and occur primarily in children and are rare after the age of 20 years2-
4
milkmaid’s grip, spooning , pronator sign, inability to control protrusion of tongue2,3
emotional lability, changes in personality, moodiness, or a change in school performance12
Jones’s Major Criteria
Chorea
non-pruritic bright pink macules or papules that blanch under pressure and spread outwards in a circular or serpiginous pattern (snake-like)commonly on the trunk and proximal extrimities; never on facedifficult to detect in patients with dark skinfound in 3% to 5%
Jones’s Major Criteria
Erythema marginatum2,4
2National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia, An evidence-based review. 2006; Pp. 7-264WHO Expert Consultation on Rheumatic Fever and Rheumatic Heart Disease. Rheumatic fever and rheumatic heart disease: report of a WHO Expert Consultation, Geneva, 29 Oct – 1 Nov 2001. Geneva: WHO 2004. (WHO Technical Report Series No. 923).
least common (1% of patients) round, freely moveable, non painful,
vary in size from 0.5 to 2 cms usually found on the extensor surfaces
of the arms and legs
Jones’s Major Criteria
Subcutaneous nodules1-4
1Seckeler MD and Hoke TR. The worldwide epidemiology of acute rheumatic fever and rheumatic heart disease Clinical Epidemiology 2011;3:67–84.1
2 National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia, An evidence-based review. 2006; Pp. 7-26.3Kaplan EL. Rheumatic Fever. In: Fauci AS (eds). Harrison’s rheumatology. New York: McGraw-hill; 2006. Pp 105-108.4WHO Expert Consultation on Rheumatic Fever and Rheumatic Heart Disease. Rheumatic fever and rheumatic heart disease: report of a WHO Expert Consultation, Geneva, 29 Oct – 1 Nov 2001. Geneva: WHO 2004. (WHO Technical Report Series No. 923).
• 38°C or higher• occurs in almost all rheumatic attacks
Minor Criteria
Fever4,11
• arthritis and arthralgia do not occurs together• is pain usually involves large joints, may be mild or
incapacitating, without associated redness or swelling, • may be present for days to weeks
Arthralgia1,3,4
• suggests that there is a first degree of heart block
• ECG should be repeated after 1–2 months to document a return to normal. If it has returned to normal, ARF becomes a more likely diagnosis.
erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and WBC count are increase as a sign of inflammatory
Acute-phase reactants2,10
Prolonged PR interval1,2
Minor Criteria
Throat Culture 8,12
May fail to isolate the organism (60% to 70% of cases) because of the latency period between the primary infection and the development of ARF12
Throat Culture 8,12
May fail to isolate the organism (60% to 70% of cases) because of the latency period between the primary infection and the development of ARF12
Evidence of antecedent GABHS infection
Streptococcal Antibody Tests8
antistreptolysin O (ASO) and antideoxyribonuclease BStreptococcal Antibody Tests8
antistreptolysin O (ASO) and antideoxyribonuclease B
Antigen Detection Tests8
RADTs are vary in method and have high specificity but low sensitivity
Antigen Detection Tests8
RADTs are vary in method and have high specificity but low sensitivity
Polyarthritis and
Fever
Carditis Chorea
Differential
Diagnosis
Septic arthritis
(including
gonococcal)
Connective tissue
and other auto-
immune disease*
Viral arthropathy†
Reactive
arthropathy†
Innocent murmur
Mitral valve
prolapse
Congenital heart
disease
Infective
endocarditis
Hypertrophic
cardio-myopathy
Systemic lupus
erythematosus
Drug
intoxication
Wilson’s disease
Tic disorder‡
Choreoathetoid
cerebral palsy
Encephalitis
Table 3. Differential Diagnosis of ARF2
2 National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia, An evidence-based review. 2006; Pp. 7-26.
Table 3. Differential Diagnosis of ARF2 cont.
Polyarthritis and
Fever
Carditis Chorea
Differential
Diagnosis
• Reactive arthropathy
• Lyme disease• Sickle-cell anemia• Infective
endocarditis• Leukaemia or
lymphoma• Gout and
pseudogout
• Myocarditis — viral or idiopathic
• Pericarditis — viral or idiopathic
• Familial chorea (including Huntington’s)
• Intracranial tumor
• Lyme disease• Hormonal
2 National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia, An evidence-based review. 2006; Pp. 7-26.
SUMMARY
ARF is a serious condition and due to physical, mental, and undue economic burden which caused by inappropriate diagnosis of ARF, accurate diagnosis of ARF is important.
Jones’s criteria as guideline to diagnose ARF. To fulfill Jones’s criteria, either two major criteria or one major criterion and two minor criterion, plus evidence of antecedent streptococcal infection are required
clinical findings are still the major consideration in making diagnosis. However many of the clinical features of ARF are non-specific, so a wide range of differential diagnoses should be considered.
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