Rheumatic Fever and Rheumatic Heart

Disease

DR.ABDUL GHAFFAR MEMON

Associate Professor Cardiology

LUMHS HYD/JAMSHORO

Learning Objectives:

• To understand the pathogenesis of acute rheumatic fever and rheumatic heart disease

• To appreciate the burden of disease• To recognize the features of a streptococcal sore

throat• To know the treatment regimens of a streptococcal

sore throat• To be aware of secondary prevention measures

Performance Objectives:

•Examine the burden of disease within own communities•Timely recognition of a streptococcal sore throat with correct treatment•Institute secondary prevention programme•Join the global community fighting Rheumatic fever and rheumatic heart disease

What is the What is the incidence of incidence of

acute rheumatic acute rheumatic fever and fever and

rheumatic heart rheumatic heart disease?disease?

EPIDEMIOLOGY

ARF is a disease of child age group 6 – 15 yrs.

ARF uncommon < 5 yrs. And recurrence very rare after 34 yrs.

Pharyngitis

80 % viral20 %

bacterial

15% GABHS

5% other bact.

.3 – 3 % ARF

Incidence of ARF: Incidence of ARF: Population-based Population-based

StudiesStudies40

35

30

25

20

15

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USA (all ages)Martinique (<20yrs)New Zealand (<30yrs)Kuwait (5-14yrs)Iran (all ages

11

5 6 7 8 9 10Time (years)

Figure 5: Trend in I ncidence of First Attack of Acute Rheumatic Fever Over Time

What is the What is the prevalence prevalence

of rheumatic of rheumatic heart heart

disease?disease?

PATHOBIOLOGY

agent hostenviron-ment

-GABHS->100 subtype of M protein

Heart-myosinHeart valve- lamininSynovia- vimentin

Skin-keratinBrain- lysoganglioside

-Extent of immune response to pharyngitis -Genetic susceptibility

-Prior history of RF

-Over crowding-low socioeconomic status

Carapetis. Lancet 2005;366:155

What is the What is the pathogenesis of pathogenesis of acute rheumatic acute rheumatic

fever?fever?

ACUTE RHEUMATIC ACUTE RHEUMATIC FEVERFEVER

• Autoimmune consequence of infection with Group A streptococcal infection

• Results in a generalised inflammatory response affecting brains, joints, skin, subcutaneous tissues and the heart.

ACUTE RHEUMATIC ACUTE RHEUMATIC FEVERFEVER

• The clinical presentation can be vague and difficult to diagnose.

• Currently the modified Duckett-Jones criteria form the basis of the diagnosis of the condition.

RHEUMATIC HEART RHEUMATIC HEART DISEASEDISEASE

• Rheumatic Heart Disease is the permanent heart valve damage resulting from one or more attacks of ARF.

• It is thought that 40-60% of patients with ARF will go on to developing RHD.

RHEUMATIC HEART RHEUMATIC HEART DISEASEDISEASE

• The commonest valves affecting are the mitral and aortic, in that order. However all four valves can be affected.

RHEUMATIC HEART RHEUMATIC HEART DISEASEDISEASE

• Sadly, RHD can go undetected with the result that patients present with debilitating heart failure.

• At this stage surgery is the only possible treatment option.

RHEUMATIC HEART RHEUMATIC HEART DISEASEDISEASE

• Patients living in poor countries have limited or no access to expensive heart surgery.

• Prosthetic valves themselves are costly and associated with a not insignificant morbidity and mortality.

What are the What are the clinical clinical

features of features of strep sore strep sore

throat?throat?

Hallmarks of Hallmarks of STREP sore throatSTREP sore throat

• Tender lymph nodes• Close contact with infected person• Scarlet fever rash• Excoriated nares( crusted lesions) in infants• Tonsillar exudates in older children• Abdominal pain• GOLD STANDARD: POSITIVE THROAT CULTURE

Hallmarks of Hallmarks of VIRAL sore VIRAL sore

throatthroat• Coryza: runny nose or mouth ulcers

• Other family with COLD symptoms

• Evidence of another viral infection

• Itchy watery eyes

• Hoarseness and cough: non-specific

• Fever: not specific

• Red Throat: not specific

2002-03 WHO criteria for the diagnosis of RF and RHF

Diagnostic Categories Criteria

• Primary episode of rheumatic fever

• Recurrent attack of RF in a pt. without stablished RHD

• Recurrent attack of RF in a pt. with stablished RHD

• Rheumatic chorea OR insidious onset rheumatic carditis.

• 2 major or 1 major plus 2 minor plus evidence of GAS Infection.

• Same as above

• 2minor manifestation plus evidence of GAS infection.

• No other major manifestations or evidence of GAS infection

Continue….

Diagnostic categories Criteria

• Chronic valve lesion of RHD (pts presenting for first time with pure MS or mixed mitral valve disease and/or aortic valve disease.)

• Do not require any other criteria to be diagnose as having RHD

ARTHRITIS

• Manifest in the 60 – 75% of pt.• Painful, migratory, and limited to the major joint of

extremities• Inflammation in 1 joint lasts for 1-2 week and polyarthritis as

whole resolve in 1 month or less.• Tenderness out of proportion to other findings.• JACCOUD Arthropathy can be occur.• Arthritis can overlap carditis but both manifestation inversely related in severity.• Very good response to salicylates.

CARDITIS

Manifest as valvulitis – (MR and/or AR) myocarditis or pericarditis or both. 40 – 60% result in RHD Soft blowing pansystolic murmur of MR is hallmark of carditis in RF. HF due to the MR not due to myocardial involvement.

Pericarditis cause friction rub and sometime pleuritic chest pain. Myocardial inflammation can cause conduction defect and heart block.There is a linear relationship b/w severity of MR during the first episode of RF and subsequent RHD.

Continue……

In the setting of LV dysfunction or/and pericarditis

myocarditis without valvular involvement path-

ology is unlikely to rheumatic in origin.

SYDENHAM'S CHOREA

Purposeless, rapid, involuntary, nonrepetitive, jerky dance like movements. Milk maid grip. Lizard tongue. Raising of hand above the head. 5 – 35% pt. of RF Prior to puberty F>>>M, after puberty no male involvement.Risk of developing subsequent RHD is 50%.

Sydenham chorea

No residual neurological deficit per se, psychiatric disturbances occur in a small no. of pts.

Recurrence are common.PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection) also a manifestation GABHS but not associated with other features of RF.

ERYTHEMA MARGINATUM

5 – 13% of RF Begin 1-3 cm, pink to red, nonpruritic, macule or papule. Trunk and proximal limb but never on face. Central clearing in serpegious fashion Exacerbate by heat. Also can seen in sepsis, drug reaction, glomerulonephritis, JRA, Lyme disease. Occur in conjunction with milder form of carditis. May lasts for months or years.

Closer view of erythema marginatum in the same patient

SUBCUTANEOUS NODULES

0 – 8% of pt. of RF External surface of elbow, knee, ankles, knuckles, scalp and spinal process. Firm, nontender, free from attachment of underlying skin. Strongly support the severity of carditis Resolve within weeks to 1 or 2 months. Not diagnostic for rheumatic fever can seen with other autoimmune disorder.

Recommended test in case of possible acute rheumatic fever..

CBCESRCRPBLOOD CULTUREECGX-RAY CHEST PA VIEW2D-ECHOTHROAT SWAB CULTURE FOR GAS (gold standard)ASO TITRE (rising) much specific for RF

What are the What are the treatment treatment

regimens of regimens of streptococcal streptococcal sore throat?sore throat?

Treatment of ARF

First line of symptomatic therapy is antiinflammatory

agent ranging from salicylates to steroid. Naproxen can be alternative for Aspirin. Bed rest in carditis pts. Effective antibiotic treatment acutely (starting less than

10 days.)almost completely eliminates risk of the dis.

Treatment

• Drug Name :Aspirin -- Begin administration immediately after diagnosis of RF. Initiation of therapy may mask manifestations of disease.

• Adult Dose: 4-8 g/d PO divided q4-6h; maintain aspirin levels in 20-25 mg/dL range until all symptoms have resolved and APRs have returned to normal

Treatment

• Drug Name: Prednisone -- Used in carditis and CHF. High-dose prednisone is administered for 2-3 wk, then tapered over 3 wk. IV corticosteroids are reserved for fulminant cases.

• Adult Dose :40-60 mg PO qd for 2-3 wk initially, then discontinue by gradual taper over 3 wk

Treatment

• Pediatric Dose :0.05-2 mg/kg PO qd for 2-3 wk initially, then discontinue by gradual taper over 3 wk

• Contraindications

• Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease

Treatment of GABHS Infection

Benzathine 1.2 million U. One time Acutely

penicillin G IM one

Penicillin V 500 mg oral BD 10 days

Amoxicillin 500 mg oral TDS 10 days

Erythromycin 250 mg oral BD 10 days

Antibiotics

• The roles for antibiotics are to (1)initially treat GAS pharyngitis,

• (2) prevent recurrent streptococcal pharyngitis, RF, and RHD, and

• (3) provide prophylaxis against bacterial endocarditis.

Treatment

• Drug Name: Penicillin V -- DOC for treatment of GAS pharyngitis.

• Do not use tetracycline and sulfonamides to treat GAS pharyngitis. For recurrent GAS pharyngitis, a second 10-d course of same antibiotic may be repeated.

Treatment• Drug Name: Benzathine /procaine

penicillin -- Adult Dose: Eradication: 1.2 million U of benzathine penicillin G or a combination of 900,000 U of benzathine penicillin G with 300,000 U of procaine penicillin G IM as a single dose

Treatment

• Erythromycin -- Used for patients who are allergic to penicillin.

• Other options include clarithromycin, azithromycin, or a narrow-spectrum cephalosporin (ie, cephalexin).

• As many as 15% of penicillin-allergic patients also are allergic to cephalosporins.

Treatment

• Drug Name: Clarithromycin -- Alternate antibiotic for treating GAS pharyngitis in patients allergic to penicillin.

• Adult Dose: 250-500 mg PO bid for 10d

• Pediatric Dose: 7.5 mg/kg PO bid for 10 d

Treatment

• Drug Name: Azithromycin -- Alternate antibiotic for treating GAS pharyngitis in patients allergic to penicillin.

• Adult Dose :12 mg/kg (not to exceed 500 mg) PO qd for 5 d

• Pediatric Dose :Administer as in adults

Treatment

• Cephalexin -- Alternate antibiotic for treating GAS pharyngitis in patients allergic to penicillin.

• Adult Dose: 250-500 mg PO qid for 10d

• Pediatric Dose: 25-50 mg/kg/d PO divided qid for 10 d

Primordial prevention

1. Improvements in socioeconomic status.

2. Prevention of overcrowding.

3. Prevention of under nutrition and malnutrition.

Primary prevention

Oral penicillin is less efficacious than Penicillin IMIAnaphylaxis is extremely unusual

Effective eradication of GABHS from the pharynx

define the role of primary prevention.

Secondary Prevention Secondary Prevention Stops sore throat, prevents recurrences of Stops sore throat, prevents recurrences of

ARF and aids in regression of RHD ARF and aids in regression of RHD

Oral penicillin has been shown to be less effective than Penicillin IMIAnaphylaxis is extremely unusual

During an episode of ARF, valve changes can be minor and are still able to regress.

After recurrent episodes of ARF, thickening of subvalvar apparatus, chordal thickening and shortening and progression to permanent valve damage is evident.

Awareness ♦ Surveillance ♦ Advocacy ♦ Prevention

Secondary Secondary prevention: prevention:

DurationDurationCATEGORY DURATION OF PROPHYLAXISAll persons with ARF with no or mild carditis

MINIMUM 10 years after most recent episode or age 21

All persons with ARF and moderate carditis

MINIMUM 10 years after most recent episode or age 35

All persons with ARF and severe carditis

MINIMUM 10 years after most recent episode or age 35 and then specialist review for need to continue. Post surgical cases definitely lifelong.

Secondary Secondary prevention: specificsprevention: specifics

PENCILLIN

Secondary prophylaxis also reduces the severity of RHD.It is associated with regression of heart disease in approximately 50-70% of those with good adherence over a decade and reduces mortality.Route:BPG is most effective when given as a deep intramuscular injection.

Secondary prevention: Secondary prevention: AdherenceAdherence

• Use a 23-gauge needle- deeper is better• Local pressure to area for 10 secs• Warm syringe to room temperature• First allow alcohol to dry or use ethylchloride

spray.

How can we reduce the pain associated with IM Penicillin?

Secondary prevention: Secondary prevention: AdherenceAdherence

• Deliver injection very slowly(over 2-3mins)• Distraction techniques• Good rapport with the case, is a significant aid to

injection comfort, compliance and understanding.• Use 0.5-1ml of 1% lignocaine. Reduces pain

significantly and excellent for younger patients.

Ensuring that patients understand their disease, are informed regarding their future and receive secondary prophylaxis

EDUCATIONEDUCATIONHealth education is critical at all levels

Lack of parental awareness of the causes and consequences of ARF/RHD is a key contributor to poor adherence amongst children on long-term prophylaxis.

A.S.A.P. Programme for A.S.A.P. Programme for the Control of RHD in the Control of RHD in

Africa:Africa: Focus areas for action Focus areas for action

• Awareness raising: public, healthcare workers

• Surveillance: incidence, prevalence, temporal trends

• Advocacy: appropriate funding of the treatment and prevention programmes

• Prevention: application of existing knowledge in primary & secondary prevention

SummarySummary• Rheumatic heart disease is the only

truly preventable chronic heart condition• Primary prevention:

– Penicillin for suspected strep sore throat

• Secondary prevention– Penicillin prophylaxis