Rheumatic Fever PDF

Prepared by Dr. shaker Ahmed Alsaggaf  Etiology Acute rheumatic fever is a systemic disease of childhood,often recurrent that follows group A beta hemolytic streptococcal infection It is a delayed non-suppurative sequelae to URTI with GABH streptococci. It is a diffuse infla...

Prepared by Dr. shaker Ahmed Alsaggaf  Etiology Acute rheumatic fever is a systemic disease of childhood,often recurrent that follows group A beta hemolytic streptococcal infection It is a delayed non-suppurative sequelae to URTI with GABH streptococci. It is a diffuse inflammatory disease of connective tissue,primarily involving heart,blood vessels,joints, subcut.tissue and CNS 2 Epidemiology Ages 5-15 yrs are most susceptible Rare boys Common in 3rd world countries Environmental factors-- over crowding, poor poor hygiene sanitation, poverty, Incidence more during Rain fall ,winter & early spring 3 Pathogenesis Delayed immune response to infection with group.A beta hemolytic streptococci. After a latent period of 1-3 weeks, antibody induced immunological damage occur to heart valves,joints, subcutaneous tissue & basal ganglia of brain 4 Group A Beta Hemolytic Streptococcus Pharyngitis- produced by GABHS can lead to- acute rheumatic fever , rheumatic heart disease & post strept. Glomerulonephritis Skin infection- produced by GABHS leads to post streptococcal glomerulo nephritis only. It will not result in Rh.Fever or carditis as skin lipid cholesterol inhibit antigenicity 5  ACUTE RHEUMATIC FEVER Redness & swelling of throat & tonsils; Beefy, swollen, red uvula; Soft palate petechiae (“doughnut lesions”) Tonsillopharyngeal erythema & Sore throat: fever, exudates white draining patches on the throat & swollen or tender lymph glands in the neck  Clinical Features A. Major criteria of Rheumatic fever(ACCNE) 1· Arthritis (75%) -Usually affect big joints (e.g. knee, ankles, wrist, elbow). - Polyarticular, either simultaneous or successive. -Migratory (fleeting) form one joint to another. - Affected joint is : ~ red - hot- swollen ~with absolute limitation of movement (severely tender) - Dramatic response to salcylates. - Resolve without residuals, even without treatment, over days to few weeks. 10  Clinical Features (Contd) 2.Carditis Manifest as pancarditis(endocarditis, myocarditis and pericarditis),occur in 40-50% of cases Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ Endocarditis: Valvulitis affecting commonly mitral valve with or without aortic valve: 11 1- Mitral valve: - Leaflets oedema ~ transient mitral stenosis (Carey Combs murmer) - Leaflets destruction ~ mitral regurge. 2- Aortic valve ~ aortic regurge. Myocarditis: 1- Tachycardia out of proportion to age & fever(rarely bradycardia due to heart block) 2- Heart failure (with cardiomegaly , gallop rhythm, &muffled heart sounds ) indicates severe carditis Pericarditis: 1- Dry pericarditis: - Stitching chest pain. -Pericardial rub (on the bare area, unrelated to respiration). 2- Pericardial effusion: -Uncommon. - Dull aching pain. -Dullness outside the apex. - Distant heart sounds. -Low voltage ECG. N.B: Carditis may be silent or late onset (appear after 6 week- 6 months of onset) Clinical Features (Contd) 3.Sydenham Chorea Occur in 5-10% of cases more in girls 8-12 years (school age). occur weeks or months after strept pharyngitis so, other criteria are usually lacking. Manifestations: l- Emotional lability and personality changes. 14 2- Involuntary movements: -Spontaneous purposeless movements of limbs and facial grimace. - increase with emotional stress and decrease by sleep. - Last for months. 3- Hypotonia.  Tests for chorea: -Milk maid's grip: irregular contraction & relaxations while sequeezing examiner fingers -Extension of arm--+ spooning & pronation of hands. (choreic hand). - Wormian movements of tongue upon protrusion. - Evaluate hand writing. Clinical Features (Contd) 4.Erythema Marginatum Occur in 1% firm, mobile, painless,pea-sized,palpable nodules. size about 1 cm. Mainly over extensor surfaces of joints,spine,scapulae & scalp Associated with strong seropositivity Always associated with severe carditis 21 SUBCUTANEOUS NODULES B. Minor criteria of Rheumatic fever i. Clinical 1. Fever usually between 38.4-40 c 2. Arthralgia (can't be used as minor manifestation in presence of arthritis) ii. Laboratory 3. Prolonged P-R interval (can't be used as minor manifestation in presence of carditis) 4. Elevated acute phase reactants (ESR, C-reactive protein, leucocytosis) Anti-DNAse B test  Jones Criteria (Revised) for Guidance in the Diagnosis of Rheumatic Fever* Major Manifestation Minor Supporting Evidence Manifestations of Streptococal Infection Carditis Clinical Laboratory Polyarthritis Previous Acute phase Chorea rheumatic reactants: Increased Titer of Anti- Erythema Marginatum fever or Erythrocyte Streptococcal Antibodies ASO Subcutaneous Nodules rheumatic sedimentation (anti-streptolysin O), heart disease rate, others Arthralgia C-reactive Positive Throat Culture Fever protein, for Group A Streptococcus leukocytosis Recent Scarlet Fever Prolonged P- R interval *The presence of two major criteria, or of one major and two minor criteria, indicates a high probability of acute rheumatic fever, if supported by evidence of Group A streptococcal nfection. Recommendations of the American Heart Association 25 Exceptions to Jones criteria: 1- Rheumatic chorea 2- Late onset carditis Each can diagnose acute rheumatic fever alone in absence of other criteria and evidence of recent strept infection but after active exclusion of other causes for both. 3- Occasionally, patients with rheumatic fever recurrences (rheumatic activity) may not fulfill Jones criteria so in patients with documented chronic rheumatic heart disease diagnosis of rheumatic activity can be made in absence· of major criteria i.e minor criteria plus evidence of recent strept infection is enough to make the diagnosis. primarily prolonged PR interval Cardiomegaly  Diagnosis Rheumatic fever is mainly a clinical diagnosis No single diagnostic sign or specific laboratory test available for diagnosis Diagnosis based on MODIFIED JONES CRITERIA 30  Treatment Step I - primary prevention (eradication of streptococci) Step II - anti inflammatory treatment (aspirin,steroids) Step III- supportive management & management of complications Step IV- secondary prevention (prevention of recurrent attacks) 32 1- Prophylactic: 1ry prevention: Hygienic housing. Treat streptococcal pharyngitis: penicillin or erythromycin for 10 days. 2ry prevention: Prevent recurrence of Rheumatic fever by: * Long acting penicillin (Benzathine penicillin) - Dose : 1.2 million unit single injection, I.M every 3 - 4 weeks. -For at least 5 years after last episode for cases without carditis - For at least 10 years after last episode for cases with carditis without residuals - For life or till age of40 for cases with carditis with residuals *Alternatives: daily oral penicillin V or erythromycin (250 mg twice daily) STEP I: Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis) Agent Dose Mode Duration Benzathine penicillin G 600 000 U for patients Intramuscular Once 27 kg (60 lb) 1 200 000 U for patients >27 kg or Penicillin V Children: 250 mg 2-3 times daily Oral 10 d (phenoxymethyl penicillin) Adolescents and adults: 500 mg 2-3 times daily For individuals allergic to penicillin Erythromycin: 20-40 mg/kg/d 2-4 times daily Oral 10 d Estolate (maximum 1 g/d) or Ethylsuccinate 40 mg/kg/d 2-4 times daily Oral 10 d (maximum 1 g/d) Recommendations ofDr.Said AmericanAlavi Heart Association 34  Step II: Anti inflammatory treatment Patients with typical migratory polyarthritis and those with carditis without cardiomegaly or congestive heart failure should be treated with oral salicylates. The usual dose of aspirin is 50-70 mg/kg/day in 4 divided doses orally for 3-5 days,followed by 50 mg/kg/day in 4divided doses for 2-3 wk and half that dose for another 2-4 wk. Determination of the serum salicylate level is not necessary unless the arthritis does not respond or signs of salicylate toxicity (tinnitus, hyperventilation)develop. (tinnitus, hyperventilation) develop. Patients with carditis and more than minimal cardiomegaly and/or congestive heart failure should receive corticosteroids. The usual dose of prednisone is 2mg/kg/day in 4 divided doses for 2-3 wk,followed by half the dose for 2-3 wk and then tapering of the dose by 5mg/24 hr every 2-3 days. When prednisone is being tapered,aspirin should be started at 50mg/kg/day in 4 divided doses for 6 wk to prevent rebound of inflammation 3.Step III: Supportive management & management of complications Bed rest Treatment of congestive cardiac failure: diuretics-vasodilators-Digoxin used cautiously Treatment of chorea: - Phenobarbitone or haloperidol Rest to joints & supportive splinting 37 STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks) Agent Dose Mode Benzathine penicillin G 1 200 000 U every 3 weeks* Intramuscular or Penicillin V 250 mg twice daily Oral or Sulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral 1.0 g once daily for patients >27 kg (60 lb) For individuals allergic to penicillin and sulfadiazine Erythromycin 250 mg twice daily Oral *In high-risk situations, administration every 3 weeks is justified and recommended Recommendations ofDr.Said American Alavi Heart Association 38 Duration of Secondary Rheumatic Fever Prophylaxis Category Duration Rheumatic fever with carditis and At least 10 y since last residual heart disease episode and at least until (persistent valvar disease*) age 40 y, sometimes lifelong prophylaxis Rheumatic fever with carditis 10 y or well into adulthood, but no residual heart disease whichever is longer (no valvar disease*) Rheumatic fever without carditis 5 y or until age 21 y, whichever is longer *Clinical or echocardiographic evidence.Heart Association Recommendations of American 39 Complications of rheumatic fever 1. Congestive heart failure: 1. Acute : due to myocarditis 2. Chronic : with episodes of acute failure precipitated by: - Rheumatic activity - Infective endocarditis - Chest infections 2. Cardiomegaly: -¢- Due to: carditis or heart failure or multivalvular lesions -¢- Detected by: - Clinically : Precordial bulge, precordial and epigastric pulsations. - Chest x ray. 3. Chronic valve lesions : -¢- Carditis ,especially in recurrences, can cause permanent organic valve lesions e.g. MS, MR , AS , AR or combined valve lesions. -¢- Organic valve lesions can be complicated with pulmonary hypertension, arrhythmia, infective endocarditis, thromboembolism & shortened life span. 4. Rheumatic activity (Recurrences ): ~Suggested by: -Fever with arthritis or arthralgia. - Change in character of already existing murmur - Appearance of new murmurs. - Carditis with heart failure. - Pericardial involvement. ~ Clinical value: it can diagnose rheumatic fever attack in presence of evidence of recent antecedent streptococcal infection 5. Pulmonary hypertension: ~ Due to long standing mitral valve lesions ~ Symptoms: dyspnea, fatigue, may be syncope ~Signs: - Pulmonary pulsations(diastolic shock) - Dull pulmonary area - Accentuated S2 - Soft ejection systolic murmur over pulmonary area. - Chest xray: Dense hilar shadows& RVH. Prognosis Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection,if not on prophylactic medicines Good prognosis for older age group & if no carditis during the initial attack Bad prognosis for younger children & those with carditis with valvar lesions 42 43