1) Acute rheumatic fever (ARF) is an immune-mediated disease that follows a streptococcal infection and can lead to rheumatic heart disease (RHD). ARF is characterized by inflammation that causes symptoms like carditis, arthritis, and chorea.
2) Carditis occurs in 30-80% of ARF cases and at least 60% of untreated patients develop RHD. RHD predominantly affects the mitral valve and causes mitral regurgitation and later mitral stenosis.
3) Management of ARF and RHD includes treating streptococcal infections, symptom relief, surgery for severe RHD, and lifelong antibiotic prophylaxis to prevent recurrent ARF and progression
1) Acute rheumatic fever (ARF) is an immune-mediated disease that follows a streptococcal infection and can lead to rheumatic heart disease (RHD). ARF is characterized by inflammation that causes symptoms like carditis, arthritis, and chorea.
2) Carditis occurs in 30-80% of ARF cases and at least 60% of untreated patients develop RHD. RHD predominantly affects the mitral valve and causes mitral regurgitation and later mitral stenosis.
3) Management of ARF and RHD includes treating streptococcal infections, symptom relief, surgery for severe RHD, and lifelong antibiotic prophylaxis to prevent recurrent ARF and progression
1) Acute rheumatic fever (ARF) is an immune-mediated disease that follows a streptococcal infection and can lead to rheumatic heart disease (RHD). ARF is characterized by inflammation that causes symptoms like carditis, arthritis, and chorea.
2) Carditis occurs in 30-80% of ARF cases and at least 60% of untreated patients develop RHD. RHD predominantly affects the mitral valve and causes mitral regurgitation and later mitral stenosis.
3) Management of ARF and RHD includes treating streptococcal infections, symptom relief, surgery for severe RHD, and lifelong antibiotic prophylaxis to prevent recurrent ARF and progression
SYARIF HIDAYAT RSUD SERANG INTRODUCTION, DEFINITION, PATHOPHYSIOLOGY Streptococcus group A haemolyticus ARF RHD An immune-mediated, multisystem inflammatory disease that follows group a streptococcal infection. Characterized by tissue inflammation that gives rise to typical clinical characteristics, including carditis, valvulitis, arthritis, chorea, erythema marginatum, and subcutaneous nodules. Carditis occurs in 3080% of patients with ARF, and at least 60% of untreated patients develop chronic RHD. A c u t e
R h e u m a t i c
F e v e r
Inflammation leads to neo vascularization, which enables further recruitment of t cells, leading to granulomatous inflammation Molecular mimicry between group a streptococcal antigens and human host tissue is thought to be the basis of this cross-reactivity. Both cross-reactive anti-bodies and cross-reactive t cells are believed to have a role in the disease. Interaction between a group a streptococcal strain and a host seems to lead to an abnormal immune response and the development of autoimmunity. Predominantly affects the mitral valve and, less commonly, the aortic valve. Mitral insufficiency is the most common valvular lesion, particularly in the early stages of the disease. Mitral stenosis develops later as a result of persistent or recurrent valvulitis, although rapid progression has been described in some developing countries. Patients with mitral insufficiency can remain relatively asymptomatic for up to 10 years, as a result of compensatory left atrial and left ventricular dilatation before the onset of left ventricular systolic dysfunction. Tricuspid regurgitation can occur as a result of volume overload, usually caused by mitral stenosis. R h e u m a t i c
H e a r t
D i s e a s e
DIAGNOSTIC CRITERIA & CATEGORY FOR ARF & RHD D I A G N O S T I C
C R I T E R I A
F O R
A R F
&
R H D
1) 2 MAYOR/ 1 MAYOR + 2 MINOR MANIFESTATIONS PLUS 2) 2 MINOR MANIFESTATIONS PLUS PLUS EVIDENCE OF A PRECEDING GROUP A STREPTOCOCCAL INFECTION D I A G N O S T I C
C A T E G O R Y
F O R
A R F
&
R H D
1 2 3 4 5 CARDITIS POLYARTHITIS MIGRANS CHOREA ERYTHEMA MARGINATUM SUBCUTANEUS NODUL Commonly presents as a heart murmur; Chest pain and/or difficulty breathing may be present in more severe cases Pain and swelling in more than one large joint (ankles, knees, wrists); Often the first complaint; Usually migratory finishes in one joint, begins in another Twitchy, jerking movements and muscle weakness (most obvious in the face, hands and feet); May occur on both sides or only one side of body; More common in teenagers and females (rare after age 20); May begin up to 3-6 months after the streptococcal throat infection, and often occurs without other symptoms; Usually resolves within 6 weeks rarely (may last 6 months or more) Painless, flat pink patches on the skin that spread outward in a circular pattern; Usually occurs early, may last months, rarely lasts years; Usually on the back or front of body, almost never on the face; Hard to see in dark-skinned people Painless lumps on the outside surfaces of elbows, wrists, knees, ankles in groups of 3-4 (up to 12) ; The skin is not red or inflamed; Last 1-2 weeks (rarely more than 1 month); Nodules are more common when Carditis is also present MANAGEMENT & PREVENTION OF ARF & RHD Secondary Primary Group A Streptococcus heamolyticus infection ACUTE RHEUMATIC FEVER PRIMARY PREVENTION P R I M A R Y
P R E V E N T I O N
SORE THROAT TREATMENT (Y) VACCINE (N) 1) Oral penicillin V 500mg 23 times per day for 10 days [250mg for children], 50 mg/kg amoxicillin per day for 10 days, or 2) A single 1,200,000u [600,000u for children 27 kg] dose of intramuscular benzathine penicillin G CLINICAL GROUPS BED REST (WEEKS) MOBILIZATION (WEEKS) CARDITIS (+), ARTHRITIS (-)
> 6 > 12 Paracetamol; Salisilat : 100mg/kg in 4-5 dose (Max 6mg/d) 2weeks then 60-70mg/kg/d for 3-6weeks; Prednison : 2mg/kg/d (max 80mg/d) 2weeks tappering off 20-25% every week; Carbamazepine, valproic acid, diazepam FEVER, ARTHRITIS, CHOREA Eradication; For 10days; QID ERYTHROMYCIN Eradication; For 10days; 25-20mg/kg/d in 3 dose (max adults 50-1000mg/d) AMOXCILLIN A c u t e
R h e u m a t i c
F e v e r
DIURETICS, ACE-I/ARB, DIGOXIN, BB, A- THROMBOTIC CARDITIS (PERICARDITIS, MYOCARDITIS, ENDOCARDITIS) ACUTE RHEUMATIC FEVER RHEUMATIC HEART DISEASE RHD MORBIDITY (CHF, AF, IE, CVD) SECONDARY PREVENTION S E C O N D A R Y
P R E V E N T I O N
NO RHD Secondary prevention; 2x250mg; every day; for all people ERYTHROMYCIN Secondary prevention; 2x250mg; everyday; for all people PENICILLIN V RHD MORBIDITY (CHF, AF, IE, CVD) DEATH CLINICAL MANAGEMENT CHF MEDICATION A-COAGULANT SURGERY/ INVASIVE NON SURGERY M A N A G E M E N T
O F
E S T A B L I S H E D
R H D
SYMPTOMATIC THERAPY MONITORING INR IN A- COAGULANT TH/ FOR AF & POST REPLACEMENT SURGERY SURGERY : REPAIR OR REPLACEMENT INVASIVE NON SURGERY : BMV CHF AF IE CVD DIURETICS, ACE-I/ARB, DIGOXIN, BB, A- THROMBOTIC DIGOXIN, BB, A-THROMBOTIC, A-COAGULANT ANTIBIOTICS, OPERATION CARDIAC EMBOLI IN AF/ VEGETATIONS IN IE (CONTROLED AF, A-COAGULANT, OPERATION); NEUROLOGIST C L I N I C A L
Hematology - Onkology Medical Division Internal Departement of Medical Faculty of North Sumatera University / Haji Adam Malik General Hospital Medan 2010