Acute Rheumatic Fever & Rheumatic Heart Disease Flashcards by Iseoluwa Ojo

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.

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ARF is Now rare in developed countries but still major public health problems in developing countries.

T/F

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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

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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

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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

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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

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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

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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

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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

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Aschoff bodies

composed of swollen _______________ surrounded by __________ and __________

eosinophilic collagen

lymphocytes and macrophages

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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

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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

preceding Group A Streptococcal
infection.

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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

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List the 5 major criteria for ARF

Carditis
Arthritis
Chorea
Erythema marginatum
Subcutaneous nodules

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Difference between major criterias in low VS moderate/high risk population?

Only in arthritis

Low- polyarthritis only

Moderate/high- mono , poly arthritis, poly arthralgia

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What nullifies prolonged PR interval as a criteria

If carditis is a major criteria

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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

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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

• 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