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F

R
A

Profesor Minodora Mazur


Implicarea articulară în FRA

• Articulațiile mari
• Asimetrice
• Tip inflamator
• Poliartrită migratorie
• Artrita, care apare la 80% dintre pacienți,
implică în mod obișnuit articulații mari,
articulațiile mai mici ale mâinilor și picioarelor
sunt mai puțin implicate
FRA este o maladie acută
inflamatorie, sistemică cu
afectarea preponderentă şi
gravă a sistemului cardio-
vascular cu instalarea VPR
RUSIA

1915-18

1891- 1918- 1955 1960


1915 1942
Scarlatina FRA
Reducerea incidenţei FRA se explică prin
Creşterea nivelului de trai
Tratamentul profilactic
Instruirea pacienţilor

Majorarea incidenţei FRA în tările în curs de


dezvoltare depinde de
Nivelul scăzut de trai
Reducerea programelor de profilaxie
Destrămarea sistemului de dispensarizare
Infecţiile faringiene şi amigdaliene
de etiologie streptococică
Factori social-economici
Vârsta
este streptococul betahemolitic grupa A şi alte
grupuri recent atestate, localizate in căile
respiratorii superioare

Infecţia precede boala cu 7-20 zile

Streptococul se transmite de la bolnav pătrunzând prin


rinofaringe sau chiar prin tegumente
Realizarea FRA după o infecţie

sporadică - are lor în 0.3%


epidemică – 3% asociată cu risc
crescut de instalare a VR
S-a demonstrat ca frecvenţa
reumatismului familiar este de 6
ori mai înaltă decât cel în
populaţie – 1.4%
Tabloul clinic
Debutul FRA
• Se poate declanşa la 1-5 săptămâni
(în mediu 19 zile) de la o infecţie
streptococică faringiană
• FRA decurge cu simptomatică
nesemnificativă în 52-70% cazuri
şi cu simptome caracteristice - 1/3 cazuri.
• La 75% cazuri debutul bolii este acut
• la 25% - insidios (lent)
In perioada de stare a bolii pacienţii
prezintă

• manifestări generale: febră, astenie,


fatigabilitate, paloare, transpiraţii,
epistaxis,
• poliartrita (articulaţiilor mari)
• cardită (dispnee)
• manifstări ale SNC (corea)
• eritem marginat
• noduli subcutanaţi
Explorări paraclinice
Teste de laborator specifice şi
simptome patognomonice pentru
FRA nu sunt

Obiectivele investigatiilor de laborator


 depistarea infecţiei streptococice (nu exista
reumatism acut fără streptococ)
 evidenţierea prezenţei şi evoluţiei inflamaţiei
reumatismale (PCR, VSH, Ig, acid sialic)
 diagnosticul afectării sistemice în special a
carditei/valvulitei –auscultativ, ECG şi EcoCG
Testele de evidenţiere a inflamaţiei
reumatismale sunt nespecifice, dar
permit obiectivarea procesului
inflamator şi supravegherea evoluţiei
bolii

Electrocardiograma este modificată în 95% din cazuri


- tulburari de ritm (extrasistole)
- tulburari de conducere - bloc AV gr.I
- supradenivelarea segmentului ST şi aplatisarea T sau
unda T negativă (miocardită/pericardită)
•- modificări ale siluetei
cardiace - cardiomegalia

•- stază în plămâni

•- pleurezii
Presentation
• Usually, a latent period of approximately 18
days occurs between the onset of streptococcal
pharyngitis and the onset of acute rheumatic
fever (ARF). This latent period is rarely shorter
than 1 week or longer than 5 weeks.
• Clinical involvement:
- Joints
- Skin
- Heart
- Central nervous system
pericardita
dilatarea cavităţilor atriilor şi
ventriculelor
regurgitarea mitrală şi aortică
(insuficienţă)
modificarea funcţiei miocardului (fracţia
de ejecţie)
Criteriile Jones, 1988
Manifestări majore

Cardită
Poliartrita
Coreea
Eritemul marginat
Noduli subcutanaţi
MANIFESTARI MINORE

Antecedente de FRA
Artralgii
Febră
Reactanţi ai fazei acute (PCR)
Prelungirea intervalului P - R
Evidenţe care presupun prezenţa
infecţiei streptococice
 Creşterea titrului anticorpilor anti-
streptococici (ASL-O)
 Izolarea streptococului grup A în culturile din
secreţiile faringiene (bacteriologic şi metoda
expres – rezultat în 24 ore)

Prezenta a 2 criterii majore sau a unui major


şi a 2 minore
indică probabilitate mare a FRA la pacienţii care
au suportat recent o faringită streptococică
Atacul acut durează 1.5-3 luni
Formele de FRA cu cardită 3-6 luni
5 % cazuri se prelungeste peste 6 luni
benefic la bolnavii fără cardită
vindecarea survine în 44 - 46% bolnavi

Pronostcul este modest la bolnavii cu cardită.


La copii instalarea valvulopatiilor reumatismale este în 40%
Cardita severă presupune frecvenţă mai mare a afecţiunilor
valvulare
La adolescenţi (15-17 ani) în 33% se instalează valvulopatii
după primul puseu FRA
La 18-21 ani - 20% cazuri
Recurenţele FRA cresc riscul instalării valvulopatiilor
reumatismale
La o parte de pacienţi se depistează VR fără anamnestic de FRA
(formă fără clinică - latentă)
1. Frecvenţa înaltă a formelor
latente a FRA asimptomatice - Modificarea
1/2 din bolnavii cu valvulopatii sindromului
nu constată puseu FRA în articular
antecedente
Afectarea
2. Majorarea limitelor de vârstă articulaţiilor mici a
a pacienţilor cu primul puseu de mâinilor şi
FRA picioarelor
rar se afectează copii de vârsta Evolutie benignă
preşcolara, mai frecvent
adoliscenţii 15-17 ani şi vârsta
tânără 18-21 ani
Antibioticul de elecţie rămâne grupa
penicilinei, streptococul fiind sensibil
la penicilina
Profilaxia vizează tratamentul
amigdalitelor prin eradicarea infecţiei
(profilaxia primară)
şi profilaxie secundară - recurenţelor
Eradicarea infecţiilor streptococe
administrarea penicilinei 2 – mln 10 zile la 6-8 ore
In alergie la peniciline
Eritromicină (macrolide) 40 mg la kg/corp/zi la copii
şi 1 gr / zi la adulţi
Nu se recomandă grupul tetraciclinei şi a
sulfanilamidelor
După 10 zile de tratament bolnavul este inclus în
programul de profilaxie a recidivelor cu Benzatin
Penicilină (Retarpen 2.4 MEGA)
În formele minore de cardită fără IC şi pericardită
Aspirina 50 mg/kg/corp/zi după mese în patru prize 6-
12 săptămâni - 2 gr.

În formele medii şi severe de cardită cu


cardiomegalie, insuficienţă cardiacă şi pericardită
- Diclofenac 100 mg
- Movalis 15 mg
- Nimesil 100 mg
Prednisolon 20 mg / zi 2-3 săptămâni cu
reducerea dozelor sau suspendare
Tratamentul IC, repaus 3-6 saptamani, regim
dietetic hiposalin
Tratamentul randomizat şi supravegherea pts cu
FRA 20 ani

25 Valvolopatii
Efecte adverse
20

15

10

0
Prednisolon Indometacina Voltaren

Efecte adverse: HTA, cefalee, vertijuri, gastropatii, cuşingoid,


acne, suprapondere
Istoriile Înţelept
Ştiinţele naturii Pur
Logica Grav
Retorica Inteligent
Scrierea Profund
Discuţiile Pregătit
Approapch to diagnosis of
arthritis, including ARF
Essential orientation
• Musculoskeletal (MK) problems present
varied challenges to the clinician
• Easy of diagnosis does vary from self-
evident to doubtful
• The TWO keys of diagnosis of MK
complaints are to:
1. take a careful history, and…
2. examine the joints carefully in order to
determine the anatomic structures involved
Always be mindful of diversity of
aetiologies
• Joint pain can be caused by diverse
processes, including:
- Inflammation
- Cartilage degeneration
- Crystal deposition
- Infections
- Trauma
• This highlights the point: The differential
Evaluation of patient with arthritis
Diagnostic clues from historical features
Understanding Patho-physiologic
basis of joint symptoms
• If the pain is stemming from the joint,
THREE broad categories of joint disease
must be differentiated:

1. Inflammatory arthritis
2. Non-inflammatory arthritis
3. Arthralgia
Usefulness of signs of inflammation in
differential diagnosis of painful joints
• Inflammatory arthritis
- Is characterized by inflammation affecting joint
structures, such as the synovium, synovial cavity, and
enthesites
- With inflammatory joint disease:
Pain both at rest and with motion
Pain is worse at rest that at the end of usage
Stiffness is present upon walking and typically lasts 30-60
minutes or longer
Joint swelling is related to synovial hypertrophy, synovial
effusion, and/or inflammation of peri-articular structures
Usefulness of signs of inflammation in
differential diagnosis of painful joints
• Non-inflammatory arthritis
- Results primarily from alterations in the structure or mechanics
of the joint
- The joint disease may occur as a result of degenerative,
traumatic or mechanical damage
- With non-inflammatory joint disease:
Pain occurs mainly or only during motion and improves quickly
with rest
Stiffness is experienced briefly (e.g. 15 min) upon walking in the
morning or following periods of inactivity
Swelling results from formation of osteophytes or from soft tissue
swelling related to synovial cysts, thickening or effusion
Usefulness of signs of
inflammation in differential
diagnosis of painful joints
• Arthralgia
- Apart from joint tenderness, no
abnormalities of joint can be identified
- May be due to an early rheumatic syndrome
whose clinical signs are not yet apparent
Different diagnostic clues from
historical features
1. Temporal pattern of arthritis:
a. Onset of symptoms – Abrupt or insidious
• Abrupt onset:
- Joint symptoms develop from minutes to
hours
- May occur in trauma, crystal arthritis, or
infection
• Joint symptoms develop over weeks to
months
Different diagnostic clues from
historical features
1. Temporal pattern of arthritis:
b. Duration of symptoms – Acute and chronic
• Acute is less than 6 weeks in duration
(septic arthritis, gout, trauma)
• Chronic is 6 or more weeks in duration
(rheumatic fever, rheumatoid arthritis,
systemic lupus erythematosus,
osteoarthritis)
Different diagnostic clues from
historical features
2. Patterns of joint involvement
• Migratory: inflammation persists for only a
few days in each joint (acute rheumatic
fever)
• Additive or simultaneous: inflammation
persists in involved joints as new ones
become affected (rheumatoid arthritis)
• Intermittent: episodic involvement occurs,
with intervening periods free of joint
Different diagnostic clues from
historical features
3. Number of involved joints
Monoarthritis: involvement of one joint
Oligoarthritis: involvement of 2-5 joints
Polyarthritis: involvement or 6 or more joints
4. Symmetry of joint involvement
Symmetric arthritis: is characterized by
involvement of the same joints on each side of
the body (rheumatoid arthritis, SLE)
Different diagnostic clues from
historical features
5. Distribution of affected joints
• The distal inter-phalangeal joints of the
fingers are usually involved in psoriatic
arthritis, gout, osteoarthritis
• Joints of the lumbar spine are typically
involved in ankylosing spondylitis
6. Distinctive types of MK involvement
• E.g., Spondyloarthropathy involves
enthuses, leading to:
Different diagnostic clues from
historical features
7. Extra-articular manifestations
• Constitutional symptoms suggest un underlining systemic disorder and are not
expected in patients with degenerative joint disease
• Skin lesions may be present and may indicate the specific diagnosis of a
number of rheumatic diseases, e.g., SLE, dermatomyositis, acute rheumatic
fever
• Ocular symptoms or signs (episcleritis, anterior uveitis, conjunctiovitis) in
reactive arthritis
Evaluation of a patient of
arthritis
Diagnostic clues from Physical evaluation
The musculoskeletal examination
• Helps distinguish joint inflammation
from joint damage
• Helps elucidate:
- Site of involvement (synovitis, enthesitis,
bursitis)
- The distribution of joint involvement
Differential diagnostic clues from
the examination
1. Signs of inflammatory joint disease
- Synovial hypertrophy (the synovial
membrane normally is too thin to palpate)
- Joint effusions (synovial inflammation,
trauma)
- Pain with motion
- Limited range of motion
- Erythema and warmth
Differential diagnostic clues from
the examination
2. Signs of degenerative joint disease
- Bony overgrowth of the joints (osteophytes)
– Heberden and Bouchard nodules
- Limited range of motion
- Crepitus (a palpable or audible grating
sensation is produced during motion of the
joint)
- Joint deformity
Differential diagnostic clues from
the examination
• Look at:
1. Affected and contra-lateral joint
2. Skin and nails (SLE rash, rheumatic fever
rash, psoriasis)
3. Eye (conjunctivitis, uveitis, dryness)
4. Genitalia (ulceration, balanitis, dischange)
5. Mouth (ulceration)
Skema of MS examination
• LOOK (at rest and during movement for
posture, deformity, swelling, muscle
wasting)
• FEEL (tenderness, swelling, muscle spasm,
crepitus, temperature)
• MOOVE (active and passive). Assess range
and stability, presence of pain
Evaluation of a patient with
arthritis
Diagnostic clues from Laboratory and other tests
Investigations for joint disorders
• Blood tests
• Imaging of bones and joints
• Synovial fluid analysis or/and synovial
biopsy
Diagnostic clues from Laboratory
tests
• Inflammatory arthritis
- High ERS
- High C-reactive protein
- Rheumatoid factor and
cyclic citrulinated peptide
- Autoantibodies (ANAs,
anti-DNA) for systemic
diseases
Diagnostic clues from imaging
studies
• Plain radiography
• CT scan
• MRI
• Joint ultrasound
Diagnostic clues from Synovial
fluid studies
• Synovial fluid analysis
- This test is used to broadly characterize the type of
arthritis (to identify cristals, and to establish the
diagnosis of septic arthritis and cristal induced
synovitis)
- Synovial fluid types are classified as:
Normal
Non-inflammatory
Inflammatory
Septic
Hemorrhagic
Definition and incidence
• Acute rheumatic fever (ARF) is a sequela of
streptococcal infection—typically following
2 to 3 weeks after group A streptococcal
pharyngitis—that occurs most commonly in
children and has rheumatologic, cardiac,
and neurologic manifestations.
• The incidence of ARF has declined in most
developed countries, and many physicians
have little or no practical experience with
Ethiology
• Although the mechanism by which
streptococcal organisms cause disease is not
entirely clear, overwhelming epidemiologic
evidence suggests that ARF is caused by
streptococcal infection, and recurrences can
be prevented with prophylaxis.
• Strains of group A streptococci that are
heavily encapsulated and rich in M protein
(signifying virulence in streptococcal
Epidemiologic data
• n the last decade, an increase in the
incidence of ARF was observed in Slovenia,
in south-central Europe. From 2008 through
2014, the estimated annual incidence of
ARF was 1.25 cases per 100,000 children.
• As many as 20 million new cases of ARF
occur each year. The introduction of
antibiotics has been associated with a rapid
worldwide decline in the incidence of ARF.
Presentation
• Usually, a latent period of approximately 18
days occurs between the onset of streptococcal
pharyngitis and the onset of acute rheumatic
fever (ARF). This latent period is rarely shorter
than 1 week or longer than 5 weeks.
• Clinical involvement:
- Joints
- Skin
- Heart
- Central nervous system
Joint involvement
• Migratory polyarthritis
• Large joints
• Assymetric
• Inflammatory type
Arthritis, which occurs in 80% of patients,
usually involves multiple large joints,
particularly the knees, ankles, elbows, and
wrists. Hips and smaller joints of hands and
feet are less commonly involved.
• SNC involvement
Sydenham chorea
- rapid, irregular, aimless involuntary
movements of the arms and legs, trunk, and
facial muscles
• Skin involvement
- Erythema marginatum
- Subcutaneous nodules
Cardiac involvement
• Suspicious signs for carditis include new or
changing valvular murmurs, cardiomegaly,
congestive heart failure, and/or pericarditis.
Nearly 60% of patients with carditis
develop isolated mitral valve involvement,
followed in prevalence by combined mitral
and aortic valve involvement.
Diagnosis – Jones criteria
Jones criteria for the diagnosis of initial ARF are the presence of
two major manifestations or one major and two minor
manifestations. For recurrent ARF, the criteria are two major
manifestations, one major and two minor manifestations, or three
minor manifestations.
• Major manifestations comprise the following:
Carditis, clinical and/or subclinical (ie, detected by
echocardiography)
Arthritis
Chorea
Erythema marginatum
Subcutaneous nodules
Diagnosis – Jones criteria
Minor manifestations in low-risk populations comprise the following:
• Polyarthralgia
• Fever ≥38.5°C
• Acute phase reactions: Erythrocyte sedimentation rate (ESR) ≥60 mm
in the first hour and/or C-reactive protein (CRP) level ≥3.0 mg/dL
• Prolonged PR interval, after accounting for age variability (unless
carditis is a major criterion)
Minor manifestations in moderate- and high-risk populations comprise the
following:
• Monoarthralgia
• Fever ≥38°C
• ESR ≥30 mm/h and/or CRP ≥3.0 mg/dL
• Prolonged PR interval, after accounting for age variability (unless
carditis is a major criterion)
Laboratory studies

• Elevated or rising streptococcal antibody


titer; a rise in titer is better evidence than a
single titer result
• A positive throat culture for group A β-
hemolytic streptococci
• A positive rapid group A streptococcal
carbohydrate antigen test in a child whose
clinical presentation suggests a high pretest
probability of streptococcal pharyngitis
Chest Radiography

• A chest radiograph may reveal


cardiomegaly.
Echocardiography with Doppler

• Echocardiography is the gold standard for


diagnosis of rheumatic heart disease
Medical Care

• Treatment strategies for acute rheumatic


fever (ARF) can be divided into the
following:
• Management of the acute attack
• Management of the current infection
• Prevention of further infection and attacks

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