ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE
Post on 21-Apr-2017
104 Views
Preview:
Transcript
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
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
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
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
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)
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
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
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
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
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
11
Major criteria
(1) Migratory poly arthritis (2) Pancarditis(3) Chorea(4) Sub cutaneous nodules(5) Erythema marginatum
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
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
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
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
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
17
Thick valves, small vegetations Fish mouth mitral valve opening
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
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
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
21
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
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
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
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
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
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
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
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
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
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)
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
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
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
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
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
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)
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
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
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
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
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%
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
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
45
THANKS FOR UR PATIENCE
top related