45
ACUTE RHEUMATIC FEVER & RHEUMATIC CARDITIS Dr. Murtaza Kamal MBBS, MD, DNB Division of Pediatric Cardiology Department of Pediatrics Safdarjung Hospital & VMMC, New Delhi DOP- 06/08/2016 1

ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

Embed Size (px)

Citation preview

Page 1: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

1

ACUTE RHEUMATIC FEVER &

RHEUMATIC CARDITIS

Dr. Murtaza KamalMBBS, MD, DNB

Division of Pediatric CardiologyDepartment of Pediatrics

Safdarjung Hospital & VMMC, New DelhiDOP- 06/08/2016

Page 2: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

2

OBJECTIVES

To know about the epidemiology of the disease

To understand the pathogenesis of rheumatic heart disease

To know about the clinical features: cardiac & non-cardiac manifestations

To learn about the laboratory studies of RHD To understand the principles of management

Page 3: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

3

ACUTE RHEUMATIC FEVERAutoimmune consequence of infection with

Group A streptococcal infectionResults in a generalised inflammatory response

affecting brains, joints, skin, subcutaneous tissues, heart, respiratory system, vessels, serosal membranes, tendons and fascial sheaths

Clinical presentation can be vague and difficult to diagnose

Currently, the Jones criteria form the basis of the diagnosis of the condition

Page 4: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

4

Epidemiology • Non suppurative complications of group A

streptococcal pharyngitis• Certain serotype of GAS (M type 1,3,5,6,18,24)• A delayed immune response caused by antibody

cross reactivity that can involve the heart, joints, skin and brain

• Latent period of 1-3 weeks• Gram positive cocci rich in M protein is a

virulence factor

Page 5: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

5

Epidemiology• Skin infection does not causes rheumatic fever

or carditis because skin lipid cholesterol inhibit antigenicity.

• Incidence 5.3/1000 in Indian population* • Incidence of RF following streptococcal throat

infection is 0.3%*• Commonest age group 5- 15 yr• First episode rare before 3 yr and after 30 yrs• Male and female both equally affected *(ICMR survey result)

Page 6: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

6

Epidemiology• Mitral valve disease and chorea are more

common in girls• Aortic valve involvement more common in boys• Poor socioeconomic condition, unhygienic living

conditions, overcrowded household predispose to streptococcal infections

• Common in tropics and subtropics• Common in colder months

Page 7: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

7

Patho-physiology• The cytotoxicity theory- GAS toxin produces

enzyme streptolysin O

• The immune mediated theory- Immunological cross reactivity between the GAS components and mammalians tissue

• M protein M1,M5,M6, M19 share epitopes with human tropomyosin and myosin

Page 8: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

8

Patho-physiology

• Infection leads to rheumatic fever several weeks after the sore throat has resolved

• The organism spreads by direct contact with oral or respiratory secretions, and spread is enhanced by crowded living conditions

• Patients remain infected for weeks after symptomatic resolution of pharyngitis and may serve as a reservoir for infecting others

Page 9: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

9

Patho-physiology

Fibrinoid degeneration of connective tissue Inflammatory edemaInflammatory cell infiltration and proliferation of

specific cells resulting in formation of ASHCOFF NODULES

• Resulting in-– Pancarditis in heart– Arthritis in joints– Nodules in subcutaneous tissue– Chorea

Page 10: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

10

Clinical presentation• Modified Jones criteria (revised in 1992) provide

guidelines for the diagnosis of rheumatic fever• The Jones criteria require the presence of: 2 major

Or 1 major and 2 minor criteria

• At least one essential criteria must be there in diagnosis of rheumatic fever

• A diagnosis of rheumatic heart disease is made after confirming antecedent rheumatic fever

Page 11: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

11

Major criteria

(1) Migratory poly arthritis (2) Pancarditis(3) Chorea(4) Sub cutaneous nodules(5) Erythema marginatum

Page 12: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

12

Minor crietria (1) CLINICAL CRITERIA- (A) Fever (B) Arthralgia (2) LABORATORY CRITERIA- (a) Acute phase reactants ( CRP, ESR) (b) Prolonged PR interval in ECG

Page 13: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

13

Essential Criteria

Evidences of recent streptococcal infections (1) Elevated ASLO titre (2) Positive throat swab culture (3)Rapid antigen test for group A streptococci

Page 14: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

14

ARTHRITIS• Most common manifestation. (70%)• Large joints (knee, elbow, ankle, wrist)• Poly arthritis- succession or simultaneous• Migratory in nature• Swelling, heat, redness, severe pain,

tenderness ,limitation of movement• Responds drammatically with salicylates• Subsides without residual deformity• Lasts 1-5 weeks

Page 15: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

15

CARDITIS• Occurs in 50% of patients• Tachycardia ( out of proportion to fever)• Heart murmur of MR or AR or both• Pancarditis ( pericarditis, myocarditis, endocarditis)1 Endocardial- - MR or AR murmurs indicative of dilatation of valve with or without associated valvulitis - Short mid-diastolic murmur (Carey-Coombs) may be present - Changing quality of heart sounds2. Myocardial- - Tachycardia even at rest. - Arrhythmias or ectopic beats - Cardiomegaly- on physical exam, CXR or ECHO - Congestive cardiac failure – right or left sided

Page 16: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

16

CARDITIS3. Pericardial- - Pericarditis - Pericardial effusion ECG Changes- - Changing contour of P waves - Inversion of T waves - Prolongation of PR interval• Sign of CHF (gallop rhythm, cardiomegaly, distant

heart sound)• Maybe self limiting or may lead to slowly progressive

valvular deformity• Mitral valve attacked in 75% cases, aortic in 30%

( but rarely as the sole valve), tricuspid and pulmonary in < 5% cases

Page 17: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

17

Thick valves, small vegetations Fish mouth mitral valve opening

Page 18: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

18

CHOREA• Sydenham’s chorea (St vitus’ dance) in 15%• More common in prepubertal girls (8-12 yrs)• Neuro psychiatric disorder• Neurological - Choreic movement and hypotonia• Psychiatric - emotional lability, hyperactivity, separation anxiety, OCD• Begins with emotional lability replaced by

choreic movement and then motor weakness• Elevated titre of anti neuronal antibody

Page 19: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

19

ERYTHEMA MARGINATUM

• In less than 10 % cases.• Non-pruritic ,serpiginous

or annular erythematous rashes.

• Trunk and inner proximal portion of extremities

• Never seen on face• Evanescent, disappears on

exposure to cold• Shape of rings or crescents

with clear centers

Page 20: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

20

SUBCUTANEOUS NODULES• 2- 10 % of cases• Commonly in cases with recurrences• Hard, painless, nonpruritic,freely movable

swelling of 0.2 to 2 cm• Extensor surface of both legs, small joints, scalp, spine• Not transient, lasting for weeks• Are recurrent• Indistinguishable from rheumatoid nodules

Page 21: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

21

Page 22: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

22

Exception of jones criteria (1) Chorea may occurs as the only manifestation of rheumatic fever (2) Indolent carditis can be the only

manifestation who comes one month after the onset of RF

(3) Some time recurrences of rheumatic fever may not fulfill the Jones criteria

Page 23: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

23

Clinical course of disease• Only carditis can causes permanent cardiac

damage– Sign of mild carditis disappears rapidly in weeks

but severe carditis may last for 2 to 6 months• Arthritis subsides with in a few days to several week– Even without treatment does not causes permanent

damage• Chorea gradually subsides in 6-7 months or longer

and does not causes permanent neurological sequele

Page 24: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

24

LABORATORY INVESTIGATIONS• Rapid antigen detection test• Throat culture• Antistreptococcal antibodies

◦The elevated level of antistreptococcal antibodies is useful, particularly in patients that present with chorea as the only diagnostic criterion

◦333 Todd Units◦Antibody titers should be checked at 2-week

intervals in order to detect a rising titer

Page 25: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

25

LABORATORY INVESTIGATIONS• Ratio of antibodies to extracellular streptococcal

antigens rises during the first month after infection and then plateaus for 3-6 months before returning to normal levels after 6-12 months

• The anti-DNAse B has a slightly higher sensitivity (90%)-240 Todd Units

Page 26: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

26

LABORATORY INVESTIGATIONS

• Antihyaluronidase results are frequently abnormal in rheumatic fever patients with a normal level of ASO titer and may rise earlier and persist longer than elevated ASO titers during rheumatic fever

• Acute phase reactants- Both tests have a high sensitivity but low specificity for rheumatic fever

Page 27: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

27

INVESTIGATIONS• CHEST X-RAYS:

– Cardiomegaly– Pulmonary congestion– Other findings consistent with heart failure

• ECHO :– Annular dilatation– Elongation of the chordae to the anterior leaflet– A postero laterally directed mitral regurgitation jet

The left ventricle is frequently dilated in association with a normal or increased fractional shortening

Page 28: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

28

INVESTIGATIONS• ECG: Sinus tachycardia Sinus bradycardia First-degree atrioventricular (AV) block (prolongation of the PR interval) ST segment elevation may be present and is

marked most in lead II, III, aVF, and V4 -V6

Page 29: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

29

INVESTIGATIONS

• Heart catheterization- In acute rheumatic heart disease, this procedure

is not indicated

With chronic disease, heart catheterization has been performed to evaluate mitral and aortic valve disease and to balloon stenotic mitral valves

Page 30: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

30

HISTOLOGIC FINDINGSAschoff bodies:

Perivascular foci of eosinophilic collagen surrounded by lymphocytes, plasma cells, and macrophagesFound in the pericardium, perivascular regions of the myocardium, and endocardium

Anitschkow cells:Plump macrophages within Aschoff bodies

Bread and butter pericarditis: In the pericardium, fibrinous and serofibrinous exudates

Page 31: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

31

TREATMENT AND MANAGEMENTTherapy is directed towards:

Eliminating the group A streptococcal pharyngitis (if still present)Suppressing inflammation from the autoimmune responseProviding supportive treatment for congestive heart failure

Following the resolution of the acute episode, subsequent therapy is directed towards:

Preventing recurrent rheumatic heart disease (in children) Monitoring for the complications and sequelae of chronic rheumatic heart disease (in adults)

Page 32: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

32

Drugs for primary prophylaxis of acute rheumatic fever

Drugs Doses Sore throat treatment

BENZATHINE PENICILLIN GDeep IM afterSensitivity test

1.2 million unit (>27 kg)0.6 million unit (<27 kg)

Single dose

PENICILLIN (oral) CHILDREN – 250 mg QIDADULTS – 500 mg TID

10 days

AZITHROMYCIN (oral)

12.5 mg/kg/dayOnce daily

5 days

CEPHALEXIN (oral) 15-20 mg/kg/dose BD 10 days

Page 33: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

33

Prevention of rheumatic fever Primary prevention- • 10 days course of penicillin therapy for

streptococcal pharyngitis• Patient sensitive to penicillin should advise

erythromycin 20-40 mg/kg in two divide dose Secondary prophylaxis- Patient with documented history of rheumatic

fever, isolated chorea, those without evidence of rheumatic heart disease must receive prophylaxis

Page 34: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

34

SECONDARY PROPHYLAXIS• Benzathine penicillin G 1.2 million units given

intra muscularly every 21st day after sensitivity testing

• Alternative method if any reaction to penicillin: (1) Oral penicillin V 250 mg twice daily (2) Oral sulfadiazine 1 gm once daily (3) Oral sulfisoxazole 0.5 gm once daily (4) Oral erythromycin ethyl succinate 250 mg BD

Page 35: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

35

Duration of prophylaxis for rheumatic feverCategory Duration

RHEUMATIC FEVER WITHOUT CARDITIS

5 yr or until age 21 yr Whichever is longer

RF WITH CARDITIS BUT WITHOUT RESIDUAL HEART DISEASE(NO VALVULAR HEART DISEASE)

10 yr or well into adulthood Whichever is longer

RF WITH CARDITIS AND RESIDUAL HEART DISEASE(PERSISTENT VALVULAR HEART DISEASE)

At least 10 yr since last episode and last until age 40 yr Some time life long prophylaxis

Page 36: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

36

Management of rheumatic fever• Bed rest:

Duration depends on type and severity of manifestation

One week for isolated arthritisSeveral weeks for severe carditis

Full activity is allowed when ESR becomes normal• Anti -inflammatory drugs:

Mild to moderate carditis-Aspirin 90-120 mg/kg/day in 4-6 divided doses for 4-6 weeks then tapering of 75 mg /kg /day in next 2 weeks

Page 37: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

37

Management of rheumatic fever• Severe carditis- Add steroid prednisone 2 mg /kg/day in four

divided doses for 2-6 weeks (If weight > 20 kg,dose of steroid 60mg/day for 3 weeks then

50mg/day for one week then 40 mg/day for next week, then reduce dose 5mg per week

If weight <20 kg,dose of steroid 40mg/day for 2 weeks then reduce by 5 mg/week)

Page 38: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

38

Management of rheumatic fever• Arthritis-

Aspirin therapy for 2 weeks then gradually tapering over 2-3 weeks

• Treatment of CHF- (1) Complete bed rest, oxygen(2) Prednisone for severe carditis of recent

onset(3) Digoxin or furosemide if indicated

Page 39: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

39

Arthritis alone

Mild carditis

Moderatecarditis

Severe carditis

Bed rest 1-2 weeks 3-4 weeks 4-6 weeks As long as congestive heart failure present

Indoor Ambulatio

1-2 weeks 3-4 weeks 4-6 weeks 2-4 months

Prednisone 0 0 0 2-6 weeks

aspirin 1-2 weeks 3-4 weeks 6-8 weeks 2-4 months

Page 40: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

40

Management of rheumatic fever• Management of chorea-

Usually self limitingReduce physical and mental stressAnti inflammatory agents are not needed

in patient with isolated choreaFor severe cases: Phenobarbitone,

haloperidolPlasma exchangeIVIG

Page 41: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

41

PROGNOSIS OF RHEUMATIC FEVER

• Presence or absence of permanent cardiac damage

• Cardiac status at the start of treatment

• Recurrence of rheumatic fever

• Regression of heart disease

Page 42: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

42

RHEUMATIC HEART DISEASE

• Results from single or repeated attacks of RF• Rigidity and deformity of valves resulting in

stenosis or incompetence or both • Mitral valve alone in 50%• Mitral + Aortic in 25%• Pure aortic uncommon• History of RF obtained in 60%

Page 43: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

43

Treatment for patients following rheumatic heart disease (RHD)

• Preventive and prophylactic therapy is indicated after rheumatic fever and acute rheumatic heart disease to prevent further damage to valves

• Patients with rheumatic heart disease and valve damage require a single dose of antibiotics 1 hour before surgical and dental procedures to help prevent bacterial endocarditis

• Patients who had rheumatic fever without valve damage do not need endocarditis prophylaxis

Page 44: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

44

Surgical CareIndication:

Heart failure persists or worsens after aggressive medical therapy for acute RHD, surgery to decrease valve insufficiency may be life-saving

40% of patients with acute rheumatic heart disease subsequently develop mitral stenosis as adults

Procedures:Mitral valvulotomyPercutaneous

balloon valvuloplastyMitral valve

replacement

Page 45: ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

45

THANKS FOR UR PATIENCE