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DIAGNOSIS OF ACUTE RHEUMATIC FEVER

Gusti Ayu Riska Pertiwi1002005069

Faculty of Medicine Udayana University

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INTRODUCTION

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• 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).

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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

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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

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CONTENT

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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

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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.

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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.

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• 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

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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).

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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.

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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

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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).

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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).

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• 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

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• 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

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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

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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.

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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.

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SUMMARY

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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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Thank youThank you