Acute Rheumatic Fever & Rheumatic Heart Disease Flashcards
Acute Rheumatic fever (ARF)
• Definition: ARF is a/an (infectious or non infectious?) complication of
__________________________________.
• A ____________ ___________________ disorder that can involve the _______,______,________, and __________.
non infectious
Group A streptococcus (GAS) pharyngitis.
multi-organ ; inflammatory
heart, joints, skin and brain.
ARF is Now rare in developed countries but still major public health problems in developing countries.
T/F
T
Epidemiology of ARF
• Age – Can occur at any age but most cases occur between _______ years.
• Females and males are affected but some studies have found a (male or female?) preponderance.
5-15 years.
female
Recurrence of RF is common in the absence of ______________________, especially during the 1st _____ yrs. after the 1st episode.
Heart complications may be long-term and severe, particularly if _________ are involved.
maintenance of low dose antibiotics
3-5
valves
Pathogenesis of RF
• RF is a (immediate or delayed?) ______________ response to ____________________________ , and the
clinical manifestation of the response and its severity in an individual is determined by
– host ________________
– the ________ of the infecting organism
• Strep M protein types such as 1, 3, 5, 6, 14, 18, 19 and 24 have been associated with RF
– a conducive environment
Delayed ; autoimmune
Group A streptococcal pharyngitis,
genetic susceptibility
virulence
Pathogenesis of RF
• Both the __________ and _________ host defenses of a genetically vulnerable host are involved.
• The patient’s immune responses (both B- and T-cell mediated) are unable to ___________________________________________.
• T ___________ and cytokine ______ appear to be key mediators of RHD.
humoral and cellular
distinguish between the invading organism and certain host tissues (molecular mimicry)
helper 1 ; Th17
Pathogenesis of ARF
• In ARF, the cross-reactive immune response results in the clinical features of rheumatic fever, including
–__________
– ____________
– ________
– _________ manifestations
carditis
transient arthritis
chorea
skin manifestations
Pathogenesis of clinical features of RF
– carditis, due to ____________ and infiltration of _________
– transient arthritis, due to the formation of __________
– chorea, due to the binding of _________ to ___________
– skin manifestations, due to a delayed
_________________
antibody binding ; T cells
immune complexes
antibodies ; basal ganglia
hypersensitivity reaction
Pathology
• In ARF, cardiac lesions can be found in _______ layer of the heart and is hence called _________
• Characteristic ________ bodies can be seen on light microscopy following heart muscle
biopsy
any; pancarditis
Aschoff
Aschoff bodies
composed of swollen _______________ surrounded by __________ and __________
eosinophilic collagen
lymphocytes and macrophages
History in an ARF patient
History of ______________ ~ __________) before onset of symptoms.
The latent period may be as long as 2-6
months in cases of ______________
• No preceding symptoms of Strep. pharyngitis in _____% of patients.
• ±Family history of rheumatic fever-it is a
(Weakly or highly?) inheritable disease.
streptococcal pharyngitis; 3 weeks
Isolated chorea; 30
Highly
Clinical manifestation/Diagnosis of
ARF
• There is no single confirmatory test for ARF.
Instead, the diagnosis of initial or recurrent
ARF relies on patients fulfilling a set of clinical criteria.
• The ___________ criteria- Comprises of _____
major manifestations, _____ minor criteria and evidence of ___________________
revised Jones; 5
4
preceding Group A Streptococcal
infection.
Definition of populations
• Low risk population
– ARF incidence ≤___ per 100,000 school aged children or all age RHD prevalence of ≤____ per 1000 population year
• Moderate or high risk population
– Children not clearly from a low risk population.
≤2
≤1
List the 5 major criteria for ARF
Carditis
Arthritis
Chorea
Erythema marginatum
Subcutaneous nodules
Difference between major criterias in low VS moderate/high risk population?
Only in arthritis
Low- polyarthritis only
Moderate/high- mono , poly arthritis, poly arthralgia
What nullifies prolonged PR interval as a criteria
If carditis is a major criteria
Difference between minor criterias in low VS moderate/high risk population?
Low- poly arthralgia
Mod/high- mono arthalgia
Low-38.5
Mod/high- 38.0
Low-ESR >60mm/hr
Mod/high- >30mm/hr
List the minor criteria for ARF
arthralgia
ESR
Prolonged PR interval
Fever
Carditis
• Clinical - significant __________ or
____________ or ________
• Subclinical or silent carditis- Pathological _______ involvement found by ___________
without clinical signs of carditis.
• MI or AI commonly. __________ and ________________ may emerge years after the acute attack (generally at adult ages).
new murmur ; Pericarditis ; CCF
valve ; echocardiogram
Mitral stenosis ; aortic stenosis
• Most common presenting symptom of ARF is ????
Arthritis
Arthritis
• Classified as _________ of the _________ in the presence of two or more of the following:
– _________ of movement, _________ of the joint and _________ in the joint and/or _________.
swelling ; joint
limitation ; hotness ; pain ; tenderness.
Typically, the arthritis of ARF is (mildly or extremely?) painful
(Small or Large?) joints are usually affected, especially _________ and _________
Extremely
Large; knees; ankles
Polyarthritis is usually (symmetrical or asymmetrical?) and _________ but
can be _________.
Highly responsive to _________ and ___________ therapy - usually responds within _______________
asymmetrical; migratory
additive
salicylate and NSAID
3 days
Subcutaneous nodules
(Common or Uncommon?)
(Highly or Not?) specific for ARF
Appears with a ————- of ARF
Occurs in crops over the elbow, wrists, knee, ankle, spine
Uncommon
Highly specific for ARF
few weeks of ARF
Erythema marginatum
(Common or Uncommon?)
(Highly or Not?) specific for ARF
(Itch or Not itchy?) or (painful or painless
Occurs on ________ and _______, rarely _______
Comes and goes for several month
Uncommon
Highly specific for ARF
Not itchy and painless
Occurs on trunks and limbs, rarely face
Comes and goes for several month
_______ major criteria can be used alone to confirm ARF diagnosis
Syndenham’s chorea
Investigations of ARF
• Acute phase reactants: ______ and ______
•SCANS: ________,________,________
•________ swab for ________ culture
• ________________ (anti-streptolysin O
titer and anti-deoxyribonuclease B titer)
• Rapid GAS antigen test
C-reactive protein and ESR
Echocardiogram, Electrocardiogram, Chest radiograph
Throat; bacterial
Streptococcal serology
Complications of ARF
• _____________
• Heart ________
• _____________ disease
Congestive cardiac failure
block; Rheumatic heart
Rheumatic heart disease
• Definition: A _____-term _______ damage caused by either a _____________ or ___________________ of ARF.
long; cardiac
single severe episode
multiple recurrent episodes
____________ is the most common cause of acquired heart disease in children and young adults globally.
RHD
Commonest Valvular lesions in RHD is ??
MR
Mitral Regurgitation
Pathology of RHD
• In RHD, Heart Valvular damage occurs:
Mostly ________ , less commonly ________, and rarely the ________ and ________ valves.
• The cardinal anatomic changes of the valve include leaflet ________, commissural ________, and ________ and ________ of the ________.
mitral ; aortic
tricuspid ; pulmonary valves.
thickening ; fusion ; shortening
thickening ; tendinous cords.
Clinical presentation of RHD
• General
–_______: Majority.
–___________ except for ___________ or
excessive precordial activity.
CCF
Asymptomatic
palpitations
Mitral regurgitation
• Signs of MR (commonest lesion in children)
– ____________
–________ apex
–________ apex beat
–_____
–__________ loudest at the apex and
radiating to the left axilla, ±Mid diastolic murmur (Carey coombs murmur - short and does not extend into late diastole).
–_________ if pulmonary hypertension has developed.
Active precordium; Heaving
Displaced; Thrill; Pan Systolic murmur
Loud P2
Aortic regurgitation
Symptoms
• Asymptomatic
• Features of ________
• _________
• ___________
• ___________
• Features of LV failure
• Easy fatigability
• Dyspnoea
• Orthopnoea
Aortic regurgitation
Signs
• __________ pulse
• ______ pulse pressure
• ____________ apex
• _________ apex
• _____________ murmur
• Collapsing pulse
• Wide pulse pressure
• Laterally displaced apex
• Thrusting apex
• Early diastolic murmur
Investigations for RHD
• CXR- to see __________
• ECG- for ________,________
• Echo.
Cardiomegaly
Tachycardia, heart block.
Screening by __________ may seriously
underestimate disease prevalence
Recent studies including one in Lagos (Ekure et al) have shown that _________________
detects heart valve damage more reliably (10 times as many cases of RHD than
auscultation).
auscultation
echocardiography
Treatment
• Medical –
– Rx CCF
– _____________ for patients with atrial fibrillation, hx of embolization and/or mechanical prosthetic valves.
– Adequate monitoring of anticoagulant therapy.
• Surgical – __________ or valve replacement
in severe valvular heart disease.
Anti-coagulant
Annuloplasty
Prevention of ARF/RHD
• ‘Primordial prevention’ refers to the
_____________________________________________ of populations at risk of ARF and RHD.
improvement of environmental, social, and
economic conditions
Prevention
– Primary: Rx of __________ within _______ of the infection with ___________ or __________ if pt. is allergic to penicillin to reduce
incidence of RF.
– Secondary: use of ________________ to reduce the recurrence of RF in people with a history of RF or RHD and, therefore, limit disease progression and allow disease resolution.
– Tertiary: refers to _________ and ___________ treatment of the _________ of RF and RHD.
acute Strep. pharyngitis
a week; penicillin or erythromycin
antibiotic prophylaxis (Penicillin)
complications
Prevention
• A _____________ is required for effective
population-level primary prevention of RF.
• A potential global vaccine is yet to be
identified due to the widespread diversity
of S. pyogenes strains and cross reactivity of streptococcal proteins with host tissues.
• Research is ongoing.
GAS vaccine
Prognosis
•______________ is the most common
cause of death in children with ARF
• 40% of patients with acute rheumatic heart disease subsequently develop______ as adults.
Severe heart failure
mitral stenosis
