childhood most common disease in developed country

1. DR KAMRUN NAHAR
CHILD SPECIALIST

2.  It is an inflammatory condition that affects joints,
skin, heart and brain.
 Occurs after a streptococcal infection (usually caused
by Group A Beta-Hemolytic Strep (GABHS)).
Definition:
RF is a multi system non supportive inflammatory disease resulting from a
systemic immune response involving mainly the joints & heart & less
frequently CNS, skin & subcutaneous tissue, triggered by group A beta
hemolytic streptococcal infection of upper respiratory tract.
It has marked tendency to recur & this recurrence could be prevented by
continuous anti streptococcal prophylaxis.
Adequate treatment of streptococcal pharyngitis with penicillin could
prevent the first rheumatic attack.

3.  Incidence:
- at any age, mostly in between 5-15 yrs of age.
- common problem in developing country.
 Why we are concern about it?
Ans- if missed or not treated properly, it may lead to
development of rheumatic heart disease (e.g. Mitral valvular
heart diseases) and/or neurological problem (e.g. Rheumatic
chorea).

4. How Group A Streptococci infection of throat
predisposes the child to develop ARF?
 Immune mimicry –
Antibodies to streptococcal bacterial components cross react with
tissues of heart, brain and joints
 Cytotoxic theory –
GAS produce a numbers of enzymes that are directly cytotoxic for
human cells, such as Streptolysin O has direct cytotoxic effect on cardiac
cells.
 Recently proposed pathogenic hypothesis-
Binding of M protein to Collagen type IV leads to antibody response to
collagen fiber resulting in inflammation in myocardium and cardiac
valves.
Theories:

5. 1. Infection of throat/pharynx
by group- A beta hemolytic
streptococcus (Serotype-
1,3,4,5,6,18,24).
2. Poor over crowded living.
3. Genetic predisposition
4. Presence of
mucopolysaccharide blood
group substance in throat.

7.  RF usually follows 1-3 weeks after pharingitis caused by GAS.
 Streptococcus attached to pharyngeal epithelium by means of
fimbriae which are coated with lepoteichoic acid also posses a
M protein which assist pathogenesis.
 Rheumatogenic strains of GAS posses a hyalorunic acid capsule
which resist phagocytosis & this enhances their virulence.
 Streptococcus remains localized at the site of infection in throat
but their product diffuse out from pharyngeal epithelium &
encounter lymphoid cells & stimulate antibody response.
 Several streptococcus antigens react immunologically with
human tissue antigen & immune response may be decreased.
 A nimber of other immunological theories have beA nimber of
other immunological theories have been suggested e.g.—
lymphocytes affected by streptococcal products at the site of
infection activate autoimmune activity.
 Another theory- similar to serum sickness has been attributed to

8. For
Sudden onset of fever, throat pain
Pharyngo-tonsillar inflammation
Cervical lymphadenopathy
Against
Coryza, cough, conjunctivitis, hoarseness

9. For diagnosis there
are 5 major and 4
minor criteria and a
requirement of
evidence of recent
GAS infection.
Analysis of major
and minor criteria in
different ARF risk
group >>>>

11. Pancarditis

12.  What are the exception of Jone’s criteria to
diagnose a case of rheumatic fever ?
Diagnosis of RF is acceptable without following Jone’s criteria with
supportive evidence of streptococcal infection are as follows-
1. Presence of chorea if other cause have been excluded.
2. Insidious or late onset carditis with no other explanation.
3. Rheumatic recurrence.
What is rheumatic recurrence ?
Patient with documented rheumatic fever
with the presence of one major criteria,
fever, arthritis, elevated acute phase
reactant plus evidence of preceding
streptococcal infection.
It suggest presumptive diagnosis of
rheumatic recurrence.

13. Joint involvement (migratory poly arthritis): (75% cases)
 Typically large joints like Knee, ankle, hip, and elbow are most commonly
involved; asymmetric joint involvement is seen.
 Pathology - Acute painful inflammation of joint; swollen, red, hot & exquisitely
tender with usually disabling (limitation of movement) ,
 Joint pain is severe and typically migratory, [moving from one joint to another
over a period of hours], Typically not deforming in nature.
 Most of the time sore throat is seen 2-4 weeks prior to onset of joint
symptoms.
 A severely inflammed joint can become normal within 1-3 days, even without
treatment.
 If the joint involvement persists >1 or 2 days after starting salicylates (Aspirin)
it is unlikely due to ARF.
 Involvement of spines, hip joints, small joints of hand & feet are uncommon
 Joint involvement is more prominent in adults and carditis in children.

14. Inflamed knee joint

15. Carditis, Pancarditis: (50-60% cases)
 Characterised by involvement of all the 3 layers of heart (i.e. endocardium,
myocardium & pericardium)
 Sub clinical carditis ( valvulitis; Valvular inflammation) also consider as major
criteria. Mitral valve is most commonly affected followed by aortic valve. But
isolated aortic or right sided valvular involvement is uncommon.
 Early valvular damage produce regurgitation. Later the leaf thickening,
scarring and calcification result in valvular stenosis.
 Patient will present with breathlessness, palpitation,chest pain, pleuritic
central chest pain,pericardial rub, tachycardia, murmur,cardiac enlargement.
 May results CCF with pulmonary and systemic congestion.
Auscultation in carditis give the following findings
 High pitched apical holosystolic murmur radiating to axilla because of MR.
 Apical soft mid diastolic murmur (CARY COOMBS MURMUR) in mitral area due
to MS
 High pitched decresendo diastolic murmur due to aortic insufficiency at left
sternal border.
 Pericardial friction rub due to pericarditis
 Softening of the first heart sound(soft S1)
 ECG will show evidence of ST or T wave changes. P-R interval prolongation.

16. CARDITIS (BEFORE RX) CARDITIS (AFTER RX)

17. 3.Sydenhams chorea (10-15% cases)
 Manifest late after initial infection with streptococcus(6 months or so).
 Manifest as spasmodic unintentional movements & possibly altered speech.
 Characteristics of movements are –
 Milk maid grip ( irregular contraction and relaxation of muscles of fingers)
 Spooning & pronation of hands when patient’s arms are extended.
 Wormian/darting movements of the tongue upon protrusion
 Detoriation of hand writing.
 Spontaneous recovery is seen within 6 weeks.
 Associated features are –
 emotional lability
 Poor school performance
 Incoordination
 Facial grimacing
4. Erythema marginatum (Rare)
 Erythematous macular rash, characteristics - red macules which fade in centre, but
remain red in the periphery.
 Non-pruritic ;Accentuated by warming the skin.
 Occurs primarily over the trunk & extremities but Never seen on the face.

20. 5. Subcutaneous nodules (<10% cases)
 These are firm, non tender, small (0.5-2 cm), mobile lumps seen beneath the
skin [extensor surfaces of tendons] overlying the bony prominences,
particularly of the hands, feet, elbows and occiput.
 It is a delayed manifestation of ARF, usually seen 2-3 weeks after onset of
disease.
 Significance – it usually associated carditis or rheumatic heart disease.
6. Other clinical features
 Pleurisy
 Pleural effusion
 Pneumonia
 High-grade fever (39°C) is the rule.
 Acute phase reactant: ESR, CRP is elevated

22.  Aschoff’s nodule.
 Aschoff nodule is a perivascular lesion consists of
central fibrinoid area surrounded by lymphocyte,
plasma cell & basophil cells.
 Found in synovial tissue of joint & tendon,
subcutaneous tissue, Heart wall of the left ventricle,
inter ventricular septum, mitral valve, aortic valve,
pericardium, blood vessels of many organ.
 The nodule heals by fibrosis sometimes leading to
extensive myocardial fibrosis when it occurs in heart
valves along the line of closure resulting thickened &
deformed, chordae shortened, commissures are
fused resulting valvular stenosis & insufficiency.

23. What is the criteria of fever in rheumatic fever ?
Usually Temp. higher than 101-103◦F, remittent in nature, it become normal
after 2-3 weeks, without treatment.
It presents along with polyarthritis & also often associated with carditis but
not with chorea.

24. Inv plan:
1. CBC with PBF:
2. Acute phase reactants (ESR,CRP): raised
3. CXR: normal or cardiomegaly (in case of carditis)
4. ECG: prolonged P-R interval (features of 1st degree heart block)
5. Echocardiography: evidence of carditis, including changes in valve rings
1. Throat swab for C/S: group A Beta hemolytic streptococcus.
 Evidence of systemic illness manifested as –
● Leucocytosis, ● raised ESR, ● raised C reactive protein.
 Evidence of preceding streptococcal infection:
◦ Throat swab culture: group A Beta hemolytic streptococcus.
◦ Anti streptolysin o antibodies: > 200(adults), > 300(children)
Hb%, Platelet normal, WBC: leukocytosis; PBF normal
CAUTION:
Dx of ARF should not be made if elevated or increasing ASO titre but
do not fulfill jones criteria

25. ECG: Prolonged PR interval
 Prolong of P-R interval.
Normal P-R interval is 12-15
sec. (avg. 18 sec).
 It is the features of first degree
heart block.
 It measures from beginning of P
wave to beginning of QRS
complex.
 P-R interval shorten as heart
rate increase.
 During P-R interval atrial
depolarization & conduction
through AV node occurs.

27. Points RF JRA
Onset Sudden onset Insidious onset
Joints involvement Large joints-usually
asymmetric
Small joints esp.
Proximal inter-
phalangeal joints
Deformity of joints Absent Present
Fleeting arthritis
Absent
Common Rare
Muscle atrophy Dramatic Present
Response to
salicylate
Common Not impressive
Cardiac involvement Common Rare
Spleen Non palpable May be palpable

29.  Counseling
 Supportive treatment:
1. Bed rest
2. Close monitoring for any features of carditis
Specific treatment:[ must]
1. Control of infection
choice of Antibiotics – Penicillin group,
if allergic to penicillin – Erythromycin, azithromycin, clindamycin etc
2. Anti inflammatory drugs: Aspirin & steroids
3. If carditis or features of heart failure: Digoxin
4. Sydenham’s chorea: Phenobarbital (drug of choice), haloperidol,
cholrpromazine

30. Indication for-
Both initial & subsequent attack of ARF can be prevented through Penicillin
prophylaxis.
Primary prevention (prevention of initial attack):
if streptococcal sore throat: Phenoxymethylpenicllin or erythromycin
Secondary prevention (prevention of subseqeunt attack):
Benzathine penicillin (I/M), Penicillin V, erythromycin,
sulfadiazine/sulfisoxazole
Duration of prophylaxis: depends on clinical presentation

31.  Indication:
 Continuous prophylaxis is recommended in patients with well documented h/o
ARF and in those with evidence of RHD.
 Prophylaxis should be initiated as soon as ARF or RHD is diagnosed.
 To eradicate residual GAS, a full course of penicillin should be given to patients
with ARF, even if a throat culture is negative.
 Drug of choice: Benzathine Penicillin IM (BEST) Penicillin V (oral)
Others- Erythromycin, Sulfadiazine
How long?– TYPE DURATION AFTER LAST ATTACK
Rheumatic fever with carditis and
residual heart disease (persistent
valvular disease†)
10 years or until age 40 years
(whichever is longer); lifetime
prophylaxis may be needed
Rheumatic fever with carditis but
no residual heart disease (no
valvular disease†)
10 years or until age 21 years
(whichever is longer)
Rheumatic fever without carditis 5 years or until age 21 years
(whichever is longer)
*—American Heart Association evidence ratings: 1C = case studies, standard of care, or consensus opinionthat a procedure or treatment is beneficial, useful, and effective.

32. 1. Rheumatic valvular disease- Mitral valve mostly
affected, aortic & tricuspid valve may also be affected.
manifestation like – MS, MR,AS,AR
2. Infective endocarditis
3. Death from myocarditis

33. Q. Which organs are affected?
ans- Joints, heart, brain and skin
Q. Sequelae in which organ?
ans- Heart
Q. Age?
ans-5-15 years, Peak at 8 years
Q. Sex?
ans - Male = Female; Chorea is common in adolescent girls
Q. Predisposing factors?
ans- Low socio-economic status, Overcrowding,
Poverty, under nutrition & Poor hygiene
Q. Genetic predisposition?
ans- HLADR3+ : risk is more.
Serum 883 B cell Allo antigen: 12 times more risk.

34. Q. What strains?
Ans - Serotypes: M 1,3,5,6,18 & 24
Q. Time interval?
Ans- 1-5 weeks (3 weeks)
Q. Untreated throat infection; risk is?
Ans - 0.3 – 3%
Q&A

35.  Rheumatic heart disease is a preventable autoimmune disease that
claims an estimated 3,20,000 lives globally every year.
 Pathogenesis involves complex interactions between specific strains
of Streptococcus pyogenes and host immune systems.
 RHD is the most commonly acquired heart disease in people under
age 25.
 Host susceptibility and environmental determinants are key factors in
disease risk and outcomes.
Definition:
Rheumatic heart disease (RHD) is a serious and long-term consequence of acute
rheumatic fever (ARF), an autoimmune sequela of a mucosal infection
by Streptococcus pyogenes (Group A Streptococcus, Strep A).

37.  Mostly affects children and adolescents (5-15 years) rarely below 3 yrs or
above 30 yrs
 Lives in low-middle income countries, especially overcrowded region.
 Risk increases with increased incidence of strep infection,
 Pregnant women with RHD are at risk of adverse outcomes, including heart
arrythmias and heart failure due to increased blood volume putting more
pressure on heart valves. It is not uncommon for women to be unaware that
they have rheumatic heart disease until pregnancy.

38. There are 2 theories of how GAS infection damages host tissues.
a. The molecular mimicry theory is that molecules on the infecting organism are
antigenically similar to molecules on host tissues. When the host immune response
targets these molecules, both are damaged. In case of acute RF, 2
main streptococcal antigens have been implicated: the surface M protein, and
GlcNAc, the immunodominant epitope of the group A carbohydrate.
b. The “neo-antigen” theory, a more recent development, suggests that GAS organism
gains access to the subendothelial collagen matrix, where M proteins binds to the
CB3 region of type IV collagen, creating a neo-antigen that induces an autoimmune
response against collagen.
 In both theories, it is thought that the initial damage to cardiac tissues is due mainly
to antibodies, with cellular responses subsequently implicated as the immunological
cascade evolves.
 These antibodies recognize and activate valve endothelium to express adhesion
molecules like vascular cell adhesion molecule 1, allowing CD4 T cells (and others)
activated by GAS to invade the heart valve, encounter antigens, and become further
activated. Over time, tissue breakdown, partly involving autoantibodies
and complement activation, releases additional endogenous antigens such as
collagen, laminin, myosin, and tropomyosin that may also serve as autoantigens,
stimulating more CD4 T cells, which then produce Th1 and potentially Th17
cytokines, leading to further inflammation in heart valve. Over time, successive
episodes coupled to resolution leads to neovascularization and fibrosis.

40. SYMPTOMS
 Features of ARF include:
 Fever
 Painful joints especially knees
ankles, elbows and wrists
 Migratory pain; that moves
between different joints
 Fatigue
 Jerky uncontrollable body
movements called ‘chorea’
 Nodules
 Heart murmur
Symptoms of heart valve
damage associated with
RHD may include:
 chest pain or discomfort shortness of
breath specially during exertion
 swelling of stomach, hands or feet
 fatigue
 rapid or irregular heart beat


41. Mitral valve:
The valve that lets blood flow from LA to LV.
Aortic valve : separating LV from aorta.
Tricuspid valve: separating RA & RV
Pic: heart, emphasizing the heart valves.

42. Manifestation of RHD depends on which valve is affected and in which
way the valve has been damaged.
Most common forms of RHD:
 Mitral valve disease refers to excessive deposit of calcium on
MV. It results unable to open the valve completely (MS) or
opening at all. This ultimately leads to a leaky valve, commonly
referred to as MR.
 Aortic valve disease refers to excessive calcium deposits in AV. It
may results development of AS, AR, or both.
 Tricuspid regurgitation involves tricuspid valve. commonly
accompanies aortic and/or mitral valve disease.
 Atrial fibrillation: Irregular and often rapid heart rate caused by
decreased blood flow, is most often associated with MS.

44. Pic: heart, emphasizing the heart valves.

47.  Most common RHD.
 Structural changes that usually includes some loss of valvular substances and shortening and thickening of Mitral
valve leaflets are shortened because of fibrosis, which lead to dialation of mitral valve ring.
C/F:
 Asymptomatic,
 Dyspnoea on exertion
 Fatigue,
 Palpiation
 Pulse pressure is increased (small water hammer pulse)
 Heart sound:
◦ S1: soft, inaudible as it is masked by systolic murmur
◦ S2: normally split, and diminished
◦ S3: may be present due to rapid ventricular filling
◦ Added sound: Pan systolic murmur, best heard at apex and radiates with equal intensity to axilla & back, and
best heard in left decubitus position [ classical diagnostic sign]
Investigation:
 CxR: cardiomegaly, LA enlargement
 ECG: LVH & LAH may be present
 ECHO: Dilated LA, LV
 Color Doppler ECHO: Regurgitate jet into LA and assess severity of regurgitation.
Treatment: only Severe MR need Rx.
 Mild to moderate MR: well tolerated without treatment.

48. 
Incidence – rare in children

MS of rheumatic origin results from fibrosis of mitral ring, commissural
adhesions and contracture of the valve leaflets, chordae, and papillary
muscle over time.

Dyspnea – most commonly seen.

Features of pulmonary congestion: cough, recurrent bronchitis, and
pulmonary hypertension leading to haemoptysis. It may also occur due to
pulmonary infarction from embolus originating in leg veins. Systemic
embolus may also present.
o/e: pulse may be small or irregular due to AF.
◦ S1: loud, may be palpable
◦ P2: usually loud,
◦ opening snap is a high pitched metallic sound that occurs immediately
following S2
◦ Added sound: Typical murmur is low pitched rumbling mid-diastolic with
pre-systolic accentuation and localized to apex.
◦ Accompanying MR may cause a pansystolic murmur.
◦ Accompanying TR secondary to RVH can cause systolic murmur.
◦ Precordium may be prominent with palpable RV impulse.

49. INVESTIGATION of MS:

CxR: enlargement of LA and its appendage and one of the main
pulmonary artery. Lung fields are congested.
In lateral & RAO position, an enlargement of LA causes a characteristic
backward displacement of barium filled esophagus.

ECG: LAH, RVH or AF

ECHO: thickened MV leaflets with doming and dilated LA, main
pulmonary artery, RV, RA. Doppler estimate pressure gradient across
valve & PA pressure.

confirmatory for MS: Cardiac catheterization

50.  AR is less common than MR.
 Pathology: in chronic rheumatic aortic insufficiency, sclerosis of aortic valve results in distortion and
retractions of the cusps; thereby, aortic valve ring gets dilated and cusps fail to appose lightly.
 C/F:
◦ Asymptomatic till dev. cardiac failure.
◦ Palpitation is frequently a distressing symptoms
◦ If LVF developed, dyspnea appears and rapidly progress.
◦ Angina may occurs, but less common than AS.
◦ Pulse pressure is wide, as because of increased systolic & lowered diastolic BP.
◦ Apex beat is displaced latterly.
◦ Heart sound: S1 & S2 are diminished, A2 is delayed and accentuated.
◦ Added sound: high pitched early diastolic murmur immediately following S2, usually
best heard along left sternal border in 3
rd
& 4
th
interspace and radiates down the left
sternal border to the apex. Murmur can be best heard when patients sit up and leans
forward holding breath in expiration. The longer the murmur, the more severe in MR.
INVESTIGATION of AR:
 CxR: enlargement of LV
 ECG: LVH
 ECHO: Aortic valve structure and may reveal vegetation in infective endocarditis. Color flow &
Doppler estimate severity of AR.
https://www.slideshare.net/EstherMaryMathew/rheumatic-heart-disease-89497407

51. HISTORY:
1. h/o recurrent sore throat with GAS
2. Features of Johnes criteria
PHYSICAL EXAMINATION: murmur
INVESTIGATION:
1. ECG: abnormal heart rhythm, cardiomegaly
2. ECHOCARDIOGRAM: physical appearances of heart valves
Challenges
 Currently a large number of people are remain undiagnosed or diagnosed at a
late stage when damage to the heart is very severe.
 It remains the leading cause of maternal cardiac complications in pregnancy.
H/O ARF Heart – carditis, valvular heart disease

52. TREATMENT:
 No cure for RHD and the damage to heart valves are permanent.
 SUPPORTIVE TREATMENTS:
◦ For angina: Glyceryl trinitrate,
◦ Palpitations: Beta blocker
◦ Heart failure: Diuertics (to reduce pulmonary congestion), ACE inhibitors, Digoxin (to control of
ventricular rate),
 SURGERY - to replace or repair the damages valve or valves.
(Depending on damage to heart valves)
 Rx of complication: Infective endocardits & LVF
PREVENTION:
RHD is preventable by preventing rheumatic fever from occurring like doing appropriate
treatment of strep throat with appropriate antibiotics.
 If already dev. ARF - prevent additional streptococcal infections to avoid ARF

53. COMPLICATION:
1. Heart failure: can occur from either a severely narrowed or
leaking heart valve.
2. Infective (Bacterial) endocarditis: Infection in damaged heart
valves.
3. Stroke from embolism
4. Complications of pregnancy and delivery due to heart
damage
5. Ruptured heart valve: Medical emergency, must need to
replace or repair heart valve.

55.  Since 2018, WHO to launch a coordinated
global response to rheumatic heart disease
and rheumatic fever.
 The Organization is working –
◦ to develop clinical guidelines for RHD
◦ Work plan to prevent RHD and care for people
already living with it.
◦ Ensuring a steady, quality supply of benzathine
penicillin is also a key priority.