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Acute rheumatic fever presentation lecture
1. LECTURE
For the 4th year students of the Faculty
of Medicine №2
ACUTE RHEUMATIC FEVER
Lecturer:
MD, professor
Krivopustov Sergey Petrovich
Dept. of Pediatrics №2
Head of the Department:
Corresponding member of NAMS of Ukraine, professor Volosovets A.P.
2. Глава I
ВТОРЖЕНИЕ БРАВОГО СОЛДАТА ШВЕЙКА
В МИРОВУЮ ВОЙНУ
Швейк несколько лет тому назад, после того
как медицинская комиссия признала его идиотом,
ушёл с военной службы и теперь промышлял
продажей собак, безобразных ублюдков, которым
он сочинял фальшивые родословные.
Кроме того, он страдал ревматизмом
и в настоящий момент растирал себе колени
оподельдоком.
For many centuries, the term «rheumatism» was used as
the definition of all musculoskeletal pathology…
3. «Rheumatism» from Ancient Greek- «Stream, flow»
World wide known publications of Sokolsky G.I. – professor
from Moskow University and French physician Jean-Baptiste
Bouillaud. In the thirties of the XIX century they described
heart damage in patients with rheumatism. That’s why it also
called Sokolsky‘s- Buyo‘s disease.
Jean-Baptiste Bouillaud
(1796 –1881)
5. It is known that Ukrainian doctor Volkovinsky F.I., who
worked in Ostrog (Rivne region), in 1817 defended the thesis
for a degree of Doctor of Medical Science “Research about
rheumatic carditis” in Latin. The thesis was submitted to
Vilenskyi University.
6. Viktor Michailovich Sidelnikov defended his
PhD thesis “Protein spectrum of blood
plasma in the clinic of children’s
rheumatism” in 1958.
7. Chief children‘s cardiorheumatologist Ministry of
Health of Ukraine, Vice-President of Association of
Ukrainian rheumatologists, Corresponding member of
National Academy of Medical Sciences of Ukraine,
Professor Volosovets Alexandr Petrovich
8. DEFINITION
Acute rheumatic fever (ARF) – acute inflammatory disease of
a connective tissue with primary localization of process in
cardiovascular system that develops in connection with the
acute infection caused by β-hemolytic streptococcus A group
in predisposed individuals.
9. EPIDEMIOLOGY
In developing countries the incidence of ARF is more, than 50
patients per 100 000 population, in USA - 0,5 per 100 000
population (M. A. Gerber, 2011).
In Ukraine the incidence of ARF is 1 patient per 100 000
children, the prevalence is 3 patients per 100 000 children
(Ministry of Health of Ukraine, 2011).
The initial attack of ARF occurs most frequently in children
aged 5-15 years.
It is rare among children in the first three 3 years of life and
persons older than 30 years.
11. ACCORDING TO ICD-10
Acute rheumatic fever (I00-I02):
I00 Rheumatic fever without mention of heart involvement
I01 Rheumatic fever with heart involvement
I01.0 Acute rheumatic pericarditis
I01.1 Acute rheumatic endocarditis
I01.2 Acute rheumatic myocarditis
I01.8 Other acute rheumatic heart disease
I01.9 Acute rheumatic heart disease, unspecified
I02 Rheumatic chorea
I02.0 Rheumatic chorea with heart involvement
I02.9 Rheumatic chorea without heart involvement
12. ETIOLOGY
ARF is caused
by β-hemolytic streptococcus A group.
There is sufficient evidence on the relationship between
pharyngitis (tonsillitis), caused by β-hemolytic streptococcus
A group, scarlet fever and ARF.
Streptococcal skin infection usually does not cause ARF.
There are the so-called “causing ARF” strains of β-hemolytic
streptococcus A group: serotypes M 1, 3, 5, 6, 18, 24.
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15. GENETICS
Relation between HLA markers, presence of B-cell marker
D8/17 and predisposition to ARF is proved
research among twins have shown higher lever ARF in
monozygotic pairs, than in dizygotic
16. PATHOGENESIS
The modern theory of pathogenesis of
Rheumatism is toxic-immunological
theory
Toxic mechanism is due to toxic effect of
streptococcal toxins.
Mechanism of cross-reactivity means that bacterial antigens
cross-react with various target organs in the body, resulting in
molecular mimicry.
The term for development of this reaction takes usually 2 weeks.
ARF is caused by streptococcal infection, contributing factors
are age, supercooling and genetic predisposition.
medchrome.com
19. PATHOGENESIS
М protein (mucoid) is a main virulence and type specific factor.
М protein inhibits phagocytic reactions, it directly affect on
phagocytes or masks reception for components complement
and opsonins. It binds fibrinogen, fibrin and its degradation
products.
Anti M-protein antibodies provide long-term immunity, but the
presence of more, than 80 serovars of M-proteins reduce the
effectiveness of this humoral defence.
Besides, М-protein has the ability of super antigen, it causes the
synthesis of antibodies with low affinity, tolerance own tissues
violated.
20. PATHOGENESIS
Streptolysin О (oxygen sensitive) shows the ability of the
hemolysin, cause damage of cardiomyocytes.
Streptolysin S (stable) bears no antigenic load.
Hyaluronidase takes part in the destruction of connective
tissue and facilitates the movement of bacteria on its fibers.
DNAase (streptodornase) promotes DNA disintegration.
Streptokinase activate plasminogen. С5а-peptidase inhibits
the activity of phagocytes, inactivate С5а component of
complement.
22. PATHOMORPHOLOGY
Rheumatic process is cyclic.
There are four phases of pathomorphological process:
1) Mucosal swelling
2) Fibrinoid swelling
3) Prolipheration stage with development of Aschow-
Talalaevsky granulemas
4) sclerosis
Duration of this process – from 4 to 6months.
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23. If it affects the heart most often happens endomyocarditis. It
begins from valvulitis, that characterized as failure of the
connective framework with subsequent deformation. Farther
process of connective tissue disorganization leads to valve
deformation, sclerosis and development of fibrous tissue.
In most patients the mitral valve affected. Aortic valve
affected less, valves of the right heart – much less.
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25. Course of
disease
Clinical criteria
Consequences
Heart failure
Basic Additional UKR Ross
Acute Rheumatic
Fever
Recurrent
(repeated)
Rheumatic fever
Carditis
Arthritis
Chorea
Erythema
marginatum
Subcutaneous
nodules
Fever
Arthralgias
Abdominal
syndrome
Serositis
Recovering
Chronic
rheumatic heart
disease:
Without heart
valvular
defects***,
With heart
valvular
defect****
0
I
II
IIA
IIБ
III
0
I
II
III
IV
CLINICAL CLASSIFICATION OF ARF
26. USE ONLY TERM
ACUTE RHEUMATIC FEVER (ARF)
clinical-time versions of the disease, that used before
(subacute, prolonged,reccurent, latent) and division «аctive
phase» into 3 degrees lost their meaning
Recurrent rheumatic fever is now considered as a new
episode of ARF, not as relapse of first episode ARF
Chronic rheumatic heart disease – disease, characterized
by failure of heart valves as postinflammatory fibrosis of
valve leafless, acquired heart valvular disease (dilatation
and/or stenosis), which were formed after undergoing ARF
27. Major manifestation Minor manifestation
Sings, which confirm
streptococcal
infection
1) Carditis
2)Polyarthritis
3)Erythema
marginatum
4) Subcutaneous
nodules
5)Chorea
1) Arthralgias
2) Fever
3) Elevated levels of
acute-phase reactants
(ESR, С-reactive
protein)
4) Prolonged PR
interval
Detection of hemolytic
streptococcus as a
result of
bacteriological
examination of
pharyngs or Strept A
Rapid test
High titer or increase
of anti streptococcal
antibodies in
dynamics
Revised diagnostic Jones criteria for the first attack ARF
(American Heart Association, 1992)
28. Revised diagnostic Jones criteria for first attack ARF
(American Heart Association, 1992)
Presence of
2 «major» manifestations
or
1 «major» plus 2 «minor»
If there is evidence of group A streptococcal infection
Allow the diagnosis of the first attack of ARF
30. АRTHTRITIS
Present in 75% of patients
Characterized as migratory polyarthritis, that typically affect
large and medium joints (the knees, ankles, elbows,
shoulders, wrists).
Often manifest as oligoarthritis.
Main characteristics:
migratory joints damage,
Symmetrical joints damage,
Painfulness and limitation movement in the joints with
lesion of periarthricular tissues (local hyperemia, swelling,
local temperature rise).
33. CARDITIS
Present in 50-60% of all cases ARF
Characterized as pancarditis, but endocarditis (valvulitis) is
typical. Myo and/or pericarditis may or will not be presented.
Carditis in mostly cases is presented as valvulitis
Usually affects the mitral valve or there is a combined failure
of aortic and mitral valvules. Isolated failure of aortic valve or
valves of the right heart are not typical for patients wits
rheumatism.
The main implication is formation of aquired heart disease,
often insufficiently of mitral valve.
35. CARDITIS
Patients complain on slackness, fatigue, dyspnoea, pain or
discomfort in chest.
Physical findings – cardiomegaly, tachycardia, weakening I
sound and systolic vulvular murmur above the apex,
arrhythmias, congestive heart failure.
Systolic murmur is due to valvulitis with defeat of mitral
valve, has blowing timbre, it is associated with І weakening
sound, takes most of the systole, may be different in
intensity. Point of its best listening is apex of the heart, it is
usually held in the left axillary region.
39. CHOREA (CHOREA MINOR, SYDENHAM CHOREA)
Occurs in 10-15% patients with ARF
Typical for childhood age, mostly for female.
Is characterized by gradual onset of neurologic symptoms.
Child becomes irritable, occurs behavior change, deterioration
of handwriting, declining school performance.
Uncoordinated jerking movement, facial grimacing, coordination
disorders occur.
Hyperkinesis usually bilateral.
Presented involuntary movements of various muscle groups,
that aggravated by stress.
40. CHOREA (CHOREA MINOR, SYDENHAM CHOREA)
Hypotonia, for exemple symptom of «flabby shoulders» is
typical. Dysarthric speech may be present.
Stretch reflexes are increased, especially the knee jerk.
Gordon’s symptom – leg remains up for a time after a knee
jerk, occurs tonic contraction of quatriceps femoris. May
occur ankle clonus.
Coordination disorders, negative Romberg’s test, negative
finger-nose and knee-heel tests are presented.
41. CHOREA (CHOREA MINOR, SYDENHAM CHOREA)
Rheumatic chorea also called «Saint Vitus Dance».
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42. ERYTHEMA MARGINATUM
Occurs in more, than 3% patients with ARF
There are light pink macules with pale centers, which is not
accompanied by itching.
Most often appears on the trunk and proximal extremities, but
not on face. Over time erythema marginatum disappears
completely. http://emedicine.medscape.com/article/333103-overview
44. SUBCUTANEOUS NODULES
Are rare, not more than in 1% patients with ARF
Are painless nodules, up to 1 сm in diameter, are located in
fascias, aponeurosis, periosteum, around joint capsules, in
subcutaneous tissue on extensor surfaces.
Usually, subcutaneous nodules disappear completely during
1-2 months.
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46. Damage of other organs and systems in patients with ARF
occurs significantly less
lungs (pneumonitis, pulmonary vasculitis, pleuritis)
kidneys (nephritis)
gastro-intestinal tract (peritonitis)
vessels (vasculitis)
eye (iritis, iridocyclitis)
thyroid (thyroiditis)
47. To confirm streptococcal etiology apply throat swab culture
Abroad is widely spread Rapid strep test or rapid antigen
detection test, that works by detecting the presence antigen
unique to group A streptococcus.
Result are generally available in 5-15 minutes.
The test have high specifity 90-100%, but sensitivity в
зависимости от условий может колебаться от 10% до 95%
(Morandi P.A. et al., 2003).
If the rapid test is negative follow-up culture might be
performed.
49. Increasing titer of antistreptolysin О (АSО),
antistreptohyaluronidase, аntistreptokinase, аnti ДNAase.
Most often used titer of АSО. Positive ASO titer is found in
80-85% of patients with ARF.
Usually antistreptococcal titers start to rise in the end of
second week after streptococcal pharyngitis (tonsillitis),
reach of maximum of up to 3-4 weeks and remain on that
level 2-3 months and then decrease for initial level.
leukocytosis, elevated ESR, C-reactive protein, blood
seromucoid, sialic acids, DPT(diphenylamine test), alpha-1,
alpha-2 and gamma-globulines.
52. Echocardiography
In patients with valvulitis detecting thickening of mitral
valve leaflets, its «shady», hypokinesia of back leaflet,
reducing of total excursion leaflets, mitral regurgitation.
In patients with aortic valvulitis detecting
Aortal regurgitation.
Also detecting left ventricular contracility, presence of
pericarditis etc.
www.cardiocares.com
53. DIFFERENTIAL DIAGNOSIS
Rheumatic cаrditis is necessary to differentiate with viral
myo- and pericarditis, infection endocarditis, congenital
heart disease, Kawasaki disease, mitral valve prolapse,
functional heart sounds etc.
Rheumatic аrthritis – with rheumatoid arthritis, reactive
arthritis, Lyme disease, septic arthritis, tuberculosis,
psoriatic arthropathy, «growing pains» in legs etc.
54. Chorea – Tics, encephalitis different etiology, attention
deficit hyperactivity disorder, Huntington‘s chorea, Gilles de
la Tourette syndrome, Cerebral palsy etc.
Erythema marginatum – allergic rash, infections erythema
nodosum (Epstein-Barr, measles, parvovirus В19,
tuberculosis, Yersinia etc), systemic lupus erythematosus,
sarcoidosis, lymphoma and other diseases.
Subcutaneous nodules – rheumatoid nodules and others.
DIFFERENTIAL DIAGNOSIS
55. In 1998 Dr. Susan Swedo describe PANDAS syndrome
(pediatric autoimmune neuropsychiatric disorders associated
with group A streptococcal infections), which is still stay the
subject of scientific debate.
Differential diagnosis is actual, when chorea minor is the only
manifestation of ARF.
PANDAS syndrome is characterized by choreiform
hyperkinesis, obsessive movements and thoughts; relation
with group A streptococcus infection is proved.
Later scientists have questioned autoimmune etiology of
syndrome and its relation with group A streptococcus
infection. Has been shown, that patients with PANDAS
syndrome are clinically heterogeneous (Roger Kurlan et al.,
2008).
DIFFERENTIAL DIAGNOSIS
56. TREATMENT
The primary goal of treating an ARF – eradicate
streptococcal organisms and bacterial antigens, elimination
of activity of rheumatic process, preventing the formation of
aquired heart diseases.
There are few stages in treatment of ARF.
First stage – hospital.
Second – specialized cardiorheumatologic sanatorium.
In the third stage in the outpatient setting are conducted
secondary prevention.
57. TREATMENT
Children with ARF are necessary hospitalized, them
prescribed bed rest, usually for 2-3 weeks.
The regime expand, when signs of acute inflammation is
subsides. In the present of carditis bed rest should be
longer.
In children with heart failure and edema restricting cooking
salt and liquid.
Antibiotics start when diagnosis of ARF is established, its
main goal – eradication of group A streptococcus.
58. Antibiotic of choice- penicillin during 10 days.
As an alternative in patients who are allergic to penicillin
macrolides are recommended (erythromycin,
mydekamitsin, clarythromycin, josamycin, spyramycin or
roxithromycin) per os during 10 days, course of
azithromycin is shorter.
After the initial course of antibiotics, patient schould
receive long-term secondary antibiotic prophylaxis.
Antibiotic of choice for secondary prevention
BENZATINEPENICILLIN
600.000 units for children ≤ 27 kg
1,2 million units for children > 27 kg
Intramuscularly every 3- 4 weeks
59. Anti inflammatory therapy depend of the clinical variant of
the disease.
Children with typical rheumatic polyarthritis, carditis
without cardiomegaly or heart failure prescribed аcetyl
salicylic during 4 weeks.
If carditis with cardiomegaly or heart failure
developing, glucocorticosteroid hormones are prescribed.
during 2-3 weeks,
60. Therapy of moderate and severe carditis include prescribing
of digoxin, diuretics, oxygen.
It must be remembered, that cardiotoxity of digoxin
increases in case of inflammation of heart muscle.
In treating of chorea anti inflammatory drugs are usually not
used. Assign protection regime, drug of choice is
Phenobarbital.
Aminochinoline derivates in the treatment of ARF in our time
is not used.
61. PREVENTION
There are primary and secondary prevention of ARF.
Primary prevention – are complex of actions, which are
directed at the prevention of primary morbidity of ARF. They
include an effective treatment of acute inflectional disease,
caused by group A streptococcus (pharyngitis, tonsillitis,
scarlet fever).
Adequate antibiotic treatment of acute streptococcal
infections for preventing ARF has a high level of evidence
(А).
Also important are measures for preventing the spread of
streptococcal infection and increasing child’s natural
resistance to adverse environmental factors.
62. Secondary prevention – prevention of new episodes of
disease and progression of pathological process in patients
undergoing ARF.
Is a regular long-term antibiotic therapy (level of evidence: В).
Antibiotic of choice for secondary prevention
BENZATINEPENICILLIN
600 thousand units for children ≤ 27 kg
1,2 million units for children > 27 kg
Intramuscularly every 4 weeks
(in high-risk situations - every 3 weeks).
Bicillin-5 at the present time are not recommended.
In case of allergy to penicillin used macrolides.
63. Duration of secondary prevention
In patients without carditis should be continued
5 years or until patient reaches 21-years of age (the principle
«as long as possible»).
In patients with ARF, which have carditis without valvular
heart disease should be continued 10 years or until patient
reaches 21-years of age (the principle «as long as possible»).
In patients, in which has formed a valvular heart disease
should be continued up to 40 years of years, and sometimes
for life.
64. PROGNOSIS
Rheumatic arthritis and chorea have not delayed effects.
The prognosis of ARF is mainly determined by cardiac
disease (the presence of valvular heart disease, its severety,
the severety of heart failure etc)
Frequency of formation of valvular heart disease after the
first attack of rheumatism: 11-14%. (Kovalenko V.M., Nesukay
O.G., 2001)
With repeated episodes of ARF frequency is much higher -
50%, that confirm extremely importance of secondary
prevention of ARF.
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