Acute rheumatic fever

Girum asirat (MD)

June 2023
Wachemo university
Introduction
 Multi system disease resulting from autoimmune reaction to infection with
group A streptococus
 Is a disease of poverty
 Crowdedness and poor hygiene leads to increased transmission of the bacteria
 Mainly disease of children ( 5 – 14 yrs)
 Rare after 30 yrs
 Adolescents and adults affected by recurrence rather than primary episode

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Pathogenesis
Etiology : infection of the URT with group A, B- hemolytic streptococcus
- any strain has the potential to cause it
- repeated pharyngitis is required to prime the immune system before
the development of ARF
Immune reaction : Autoimmune reaction leads to damage of host tissue because
of cross reactivity between antigens of the organism and host tissue

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Clinical features
 Latent period : 3 wks ( 1- 5 wks )
 Most preceding pharyngeal infection are subclinical
Heart involvement :
60% of patients with ARF develop rheumatic heart disease
it is pancarditis
damage of heart valves is the hallmark
Mitral valve is almost always affected
Aortic valve may be involved with the mitral valve but isolated aortic valve
involvement is rare
Early valve damage leads to regurgitant murmur
With recurrent episode leaflet thickening , scarring , calcification and valvular
stenosis may develop

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Clinical features contd..
 Pericarditis , friction rub , small effusion on echocardiography
 Rarely pleuritic chest pain from adjacent involvement of the pleura
 Myocardial involvement is almost never responsible in itself for cardiac failure
but can affect electrical conduction pathways leading to PR lnterval
prolongation and softening of S1

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Clnical features contd…

Joint involvement :
-to qualify as a major manifestation joint involvement must be arthritic (
with evidence of inflammation)
-it is a migratory arthritis moving from one joint to the other in period of
hours
- it involves large joints ( ankle , knee , hip , elbow)
- arthralgia with out signs of inflammation may occur with similar pattern as
arthritis but only qualify as minor criteria
-Highly responsive to NSAIDS : joint involvement that stays more than one to
two days after starting salicylates is unlikely to be due to ARF

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Clnical features contd…

Chorea : sydenhams chorea usually occur in the absence of other manifestations

-usually in females
-abnormal body movement : eg . Darting movement of the tongue and
extremity
-usually resolve in 6 weeks

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Clnical features contd…

 Skin :
-erythema marignatum
-subcutaneous nodules
 Other features :
-fever

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Laboratory investigations
 CBC : leukocytosis
 ESR and CRP : raised
 ASO titer or anti DNAse B titer
 Throat culture
 Rapid antigen tests for streptococcus antigens
 ECG : PR prolongation
 ECHO : new or worsening regurgitant murmur
: minimal pericardial fluid

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2002/2003 WHO criteria for the
diagnosis of rheumatic fever and
rheumatic heart disease
Diagnostic categories Criteria

Primary episode of rheumatic fever 2 major or 1 major + 2 minor +

evidence of GA strept infecion

Recurrent attack of rheumatic fever 2 major or 1 major + 2 minor +

in a patient without estabilished RHD evidence of GA strept infecion

Reccurent rheumatic fever in a 2 minor + evidence of streptococcal

patient with already estabilished RHD infection
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Chorea and indolent carditis No other criteria or evidence of
strept infection is required
 Moderate or high risk
Risk category
Diagnostic Low risk
criteria contd..

Major Carditis both clinical and Carditis ...clinical and

subclinical subclinical
Polyarthritis Polyarthritis
Chorea Monoarthritis
Erythema marginatum Polyarthralgia
Subcutanous nodule Chorea
Subcutanous nodule
Erythema marginatum
Minor Fever 38.5 or more Fever more than 38
Polyarthralgia Monoarthralgia
ESR 60 mm/hr or more ESR 30MM/hr or more
CRP 3mg/dl or more
CRP more than 3mg/dl
Leuckocytosis
Leuckocytosis
Prologed PR interva unless
Prolonged PR interval carditis is used as majior
criteria
ECG : prolonged P - R 1/31/2024
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interval
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 Low risk....ARF prevalence less than or equal to 2/100,000 school age
children
All age rheaumatic heart disease prevalence less than or equal to
1/1000 population
Moderate/high risk..ARF prevalence more than 2/100,000 school age children
All age rheaumatic heart disease prevalence more than 1/1000
Population
Treatment

 No treatment for ARF prevents the development

or severity of RHD
 Antibiotics :- amoxicillin 500 mg po tid for 10
days
-benzathine penicillin G 1.2 million
international units IM stat
-erythromycin 250 mg po bid for 10 days
for penicillin allergics
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Treatment contd..

 Salicylates and NSAIDS :

- treatment of arthritis , arthralgia , and fever once diagnosis is confirmed
-of no value in treatment of carditis or chorea
-Aspirin ( ASA) 80 – 100 mg/kg/day for the first few days up to 2 weeks
When acute symptoms resolve decrease dose to 60 – 80 mg/kg/day for further 2 –
4 weeks

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Treatment contd..

 Carditis : conventional therapy for CHF

prednisolone 1-2 mg/kg/day required for only few days up to 3
weeks maximum
 Chorea : medication does not alter the duration or out come of chorea
carbamazepine , valproate , haloperidol

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Prognosis of ARF
 Untreated ARF stays for 12 weeks
 Weekly or 2 weekly follow up of ESR and CRP ( normalize within 4- 6 weeks)
 Echo after 1 month to see the progression of carditis
 60 % of patients with ARF develop rheumatic heart disease

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Prevention

Primary prevention :
ideal : eliminating risk factors for strept infection which are over
crowdedness and poor hygiene
-if sore throat is treated within 9 days of commencement ARF will be
prevented

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Secondary prevention

-Longterm antibiotic prophylaxis is required for those patients who have

Patient
sustained category
an episode of rheumatic feverDuration of prophylaxis

Patient with out proven For 5 years after last attack

carditis or 18 yrs old which over
comes late

Patient with mild MR or 10 yrs after last attack or

healed carditis till 25 yrs old which ever
comes late

More severe VHD Lifelong

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Valve surgery Life long
secondary prevention contd..

 Antibiotic for prophylaxis :

-benzathine penicillin 1.2 million IU every 2- 4 weeks
-erythromycine 250 mg po BID

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 Infective
endocarditis
Introduction
 IE is infection of the endocardial surface of the heart most commonly on the
valves.

 The prototypic lesion is vegetation which is a mass of fibrin platelets ,

microorganisms and inflammatory cells

 The intra cardiac effects of IE leads to severe valve insufficiency and CHF

 Systemic signs and symptoms also occur through different mechanisms

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Classification of IE Based on
the duration of Illness
1. Acute IE : less common and it affects healthy
native valves
- it is rapidly developing febrile disease
with destruction of valvular structure
- if untreated rapidly progresses to death
in weeks

2. Subacute IE : has insidious course with slowly

progressing valve damage
-usually occurs on already damaged
valves (eg on valves affectes by RHD)

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 CLASSIFICATION (BASED ON
PREDISPOSING RISK
1.

FACTORS)
Native valve endocarditis
community acquired

 Health care associated (nosocomial) endocarditis: defined as a diagnosis of IE made

more than 72 hours after admission in patients with no evidence of IE on admission, or IE
developing within 60 days of a prior admission when there was a risk factor for
bacteremia or any risk factor for IE during the hospitalization
2. Prosthetic valve endocarditis
3. Endocarditis in IVD abusers

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Epidemiology

 Native valve endocarditis is the commonest cause in third world countries

 IE especially develops on previously damaged valves(eg. due to RHD ,

degenerative disease )

 Prosthetic valve IE and IE in IV drug abusers are concerns of developed

countries

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Etiology

 Many species of bacteria and fungi can cause sporadic IE but most of the IE is
caused by certain type of bacteria

 Native valve community acquired IE:

-The pathogens vary with the clinical picture of the disease and the portal of
entry
-Oral cavity => Streptococcus viridans
-Skin====== => Staphylococcus
-URT========> HACEK organisms
-GIT=========== => streptococcus bovis
-GUT=========== => enterococci

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Etiology contd..

 Prosthetic valve infections : staph aureus or coagulase negative

staphylococci

 IV drug abusers : commonly Staph. aureus , but Pseudomonas aureginosa and

candida species can also be incriminated

 Nosocomial IE : staph aureus

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Pathogenesis contd..

 IE affects the mitral valve , aortic valve , combined aortic and mitral , and tricuspid
valve and rarely the pulmonic valves in the respective order

 The cardiac conditions that leads to the formation of NBTE which are the sites of
infection are MR , AS , AR , VSD and other complex congenital heart diseases

 The systemic manifestations of IE are caused due to

 embolic phenomenon from the infected thrombus
 hematogenous dissemination of infecting organisms
 immunologic reaction from ag/ ab deposition
 cytokine mediated effects

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Clinical features

 Sub acute IE :
 is caused by organisms like S . viridans , enterococci , HACEK organisms

 The clinical features of sub acute IE are non specific

 Fever is typically low grade

 is a slowly progressive process characterized by fever , fatigue , anorexia , back pain and
weight loss

 Cardiac manifestations are rare or occur late in untreated patients

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Clinical features contd

 Acute IE:
 is a much more progressive disease

 Caused by Staph aureus

 The clinical symptoms result from either embolic or intracardiac suppurative complications.

 There is an acute onset of high grade fever and chills and rapid onset of CHF

 Complications develop with in a week

 Because of its short course immunologic phenomenon are not part of acute IE

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Cardiac manifestations :
 new regurgitant murmur from valvular damage and rupture of chordae tendinae

 congestive heart failure resulting usually from the valvular abnormality or myocarditis

 extension of the infection to the adjacent myocardium leads to perivalvular abscess

which may interrupt the normal electrical impulse transmission leading to heart
blocks(arrhythmia)

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Clinical manifestation contd..

Non cardiac manifestations :

 hematogenous dissemination of the infecting organism to the skeletal system , GI ,
meninges , kidneys , skin

 arterial embolization to the brain , bowel , spleen , extremity resulting in

infarction

 immunologic complications: glomerulo nephritis , spenomegaly , oslers nodes

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CLINICAL FEATURES OF IE

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Clinical manifestation contd..

 Immunologic manifestaions of IE :
-acute glomerulonephritis
-osler’s nodes
-roth spot
-presence of rheumatoid factor

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Physical examination
 Fever
 Murmurs
 Peripheral lesions of SBE (not common to see currently because of early initiation
of antibiotics) :
petechia
splinter hemorrhage
Clubbing
Splenomegaly
Oslers nodes
Janeway lesions

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 NON CARDIAC MANIFESTATIONS OF IE

 Janeway lesions: are

macular, blanching,
nonpainful, erythematous
lesions on the palms and
soles

 Osler's nodes: are painful,

papulopustular to
violaceous nodular lesions
found in the pulp of
fingers and toes and are
seen more often in
subacute than acute
cases of IE 1/31/2024
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 PERIPHERAL MANIFESTATIONS OF IE

 Roth spots are exudative,

edematous hemorrhagic lesions
of the retina

 petechie

 Splinter hemorrhage: are

nonblanching, linear reddish-
brown lesions found under the
nail bed
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 Non cardiac manifestations

 Embolization:
 more common in patients with S. aureus IE

 vegetations >10 mm in diameter , and infection involving

the mitral valve are independently associated with an
increased risk of embolization

 skin, spleen, kidneys, skeletal system, and meninges are

commnly involved

 Stroke, Blindness, painful ischemic or frankly gangrenous

extremities, unusual pain syndromes (eg, due to splenic or
renal infarction), hypoxia (due to pulmonary emboli in
right-sided endocarditis) and paralysis (due to embolic
infarction of either the brain or spinal cord)

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 Non cardiac manifestations
 Neurologic complications:
 embolic stroke

 acute encephalopathy

 Meningoencephalitis

 purulent or aseptic meningitis

 cerebral hemorrhage (due to stroke or a ruptured mycotic aneurysm)

 brain abscess or cerebritis

 seizures (secondary to abscess or embolic infarction)

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 NON CARDIAC MANIFESTATIONS
 Mycotic aneurysm

 Renal disease
 renal infarction (due to emboli)
 drug-induced acute interstitial nephritis
 glomerulonephritis (due to deposition of immunoglobulins and
complement in the glomerular membrane)
 rarely, renal abscess can occur in patients with IE

 Metastatic abscess

 Musculoskeletal complications

 Complications of surgical and medical therapy 1/31/2024

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INVESTIGATIONS

INFECTIVE ENDOCARDITIS

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 BLOOD CULTURE
 Blood cultures should be obtained prior to antibiotic therapy

 Atleast 3 blood culture sets (2 bottles per set), separated from each
other by 1 h, should be obtained from different venipuncture sites over
24 h

 Each bottle should be inoculated with a minimum of 10 mL (and

preferably 20 mL) of blood (the estimated yield of blood cultures in
bacteremic adults increased approximately 3 percent per mL of blood
cultured)

 Blood cultures can be taken at any time; they do not need to be

obtained with the appearance of chills or fever

 From 5- 15% of patients with IE have negative blood cultures; in 1/3 -

1/2 of these cases, cultures are negative because of prior antibiotic
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exposure
 Echocardiography
 allows anatomic confirmation of IE, sizing of vegetations, detection of
intracardiac complications, and assessment of cardiac function

 TTE
 is noninvasive and exceptionally specific
 cannot image vegetations <2 mm in diameter
 detects vegetations in only 65% of patients with definite clinical endocarditis
 TTE is not adequate for evaluating prosthetic valves or detecting intracardiac
complications

 TEE
 is safe and detects vegetations in >90% of patients with definite IE
 initial studies may be false-negative in 6–18% of IE pts
 optimal method for the diagnosis of PVE or the detection of myocardial abscess,
valve perforation, or intracardiac fistulae.
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 ADDITIONAL TESTS
 CXR and ECG

 Serologic tests can be used to implicate causally some organisms that are difficult to recover by
blood culture: Brucella, Bartonella, Legionella, and C. burnetii

 An elevated ESR and/or CRP

 A normochromic normocytic anemia

 WBC count may be normal or elevated in patients with subacute presentations of endocarditis;
however, most patients with staphylococcal endocarditis have leukocytosis and some may have
thrombocytopenia

 Hyperglobulinemia, cryoglobulins, circulating immune complexes, hypocomplementemia,

elevated rheumatoid factor titers, and false positive serologic tests for syphilis all occur in
some patients

 An abnormal U/A, as manifested by microscopic or gross hematuria, proteinuria, and/or pyuria

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 RFT, CT/MRI scan 45
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DIAGNOSIS OF IE

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treatment

 Principles antimicrobial antibiotics

 Prolonged treatment
 Bactericidal
 parenteral

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 Emperic treatment
 Only should be started after 2 sets of blood culture taken

 Coverage of streptococci, staph and enterococci must be considered

 beta lactam such as penicillin + aminoglycoside, has been shown to be highly

effective in streptococcal and enterococcal endocarditis, and of equivocal
efficacy in patients with staphylococcal endocarditis

 penicillin + aminoglycoside, or ceftriaxone+ aminoglycoside for 2wks is highly

effective in streptococcus viridans endocarditis

 Nafcillin + aminoglycoside for 2wks has been shown to be effective in patients

with right-sided IE due to S. aureus .

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ANTIBIOTIC TREATEMENT OF IE

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ANTIBIOTIC TREATEMENT OF IE

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INDICATIONS FOR CARDIAC SURGICAL
INTERVENTION IN IE

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GENERAL MEASURES FOR THE PREVENTION OF IE

 Incidence of IE can be reduced by total surgical

correction of some congenital lesions, such as patent
ductus arteriosus, ventricular septal defect, and
pulmonary stenosis

 Maintaining good oral hygiene, which decreases the

frequency of bacteremia that accompanies daily
activities, is an important preventive measure

 Infections associated with bacteremia must be treated

promptly and, if possible, eradicated before the
involved tissues are incised or manipulated

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 CHEMOPROPHYLAXIS
 Prophylactic antibiotics are advised only for those patients at highest risk
for severe morbidity or death from endocarditis

 Prophylaxis is recommended only for dental procedures wherein there is

manipulation of gingival tissue or the periapical region of the teeth or
perforation of the oral mucosa (including surgery on the respiratory tract)

 Although prophylaxis is not advised for patients undergoing GI or GI tract

procedures, it is recommended that effective treatment be given to these
high-risk patients before or when they undergo procedures on an infected
GUT or on infected skin and related soft tissue

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High-Risk Cardiac Lesions for Which Endocarditis
Prophylaxis Is Advised before Dental Procedures

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Antibiotic Regimens for Prophylaxis of Endocarditis
in Adults with High-Risk Cardiac Lesions

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