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8. RHD (2)

Pathogenesis,types ,causes and diagnosis and treatment

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8. RHD (2)

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  1. Rheumatc heart disease Professor mohammed Ahmed Bamashmos Professor of internal medicine and 3

  2. Acute rheumatic fever • Rheumatic fever is an acute, immunological mediated , multisystem ( heart , joint , CNS, skin, subcutaneous tissue) affecting inflammatory disease that occurs a few weeks following an episode of group A Beta streptococcal pharyngitis • Chronic stage of RF involves all the layers of heart ( pancarditis) causing major cardiac sequelae referred to as rheumatic heart disease ( RHD).

  3. Rheumatic Heart Disease • Epidemiology a. Occurs at 5 to 15 years of age b. Develops over 1 to 5 weeks ( average 20 days) after group A streptococcal (streptococcus pyogenes) pharyngitis • Only site for infection leading to Rheumatic fever c. Risk factors for streptococcal pharayngitis (1) crowding (2) poverty (3) young age d. Recurrent RF produces chronic valvular disease

  4. Pathogenesis a. Immunie- mediated disease that follows group A streptococcal infection – sore thorat /pharyngitis b. Antibodies develop against group A streptococcal M proteins (1.) antibobies cross – react with similar protiens in human tissue ( called mimicry) - type II hypersensitivity reaction (2) cell- mediated immunity has also been implicated - type IV hypersensitivity reaction (3) nephrogenic strains of group A streptococcus lack M- protiens - never associated with Rheumatic fever c. Acute rheumatic fever ( carditis, arthritis ) 🡪 Rheumatic heart disease Chronic RF : by damage valves ( mitral , aortic) by fibrosis

  5. Histologically- • Fibrinoid degeneration is seen in the collagen of connective tissue • Aschoff nodules are pathognomonic and occur only in the heart.

  6. Clinical findings • Jones Criteria (1992 Revision) for Diagnosis of Acute Rheumatic Fever* • Major manifestations • 1. Carditis (~ 35%) • 2. Migratory Polyarthritis (~ 75%) • 3. Chorea (~10%) • 4. Erythema marginatum (10%) • 5. Subcutaneous nodules (~10%)

  7. Minor manifestations • 1. Fever • 2. Arthralgias • 3. Previous rheumatic fever or rheumatic heart disease • 4. Increased C-reactive protein (CRP) concentrations or erythrocyte sedimentation rate ( ESR) • 5. Prolonged PR interval on electrocardiogram • 6. Absolute neutrophilic leukocytosis

  8. Evidence of antecedent group A streptococcal infection 1. Positive throat culture 2. Rapid antigen test positive for group A streptococcus (ASO titre) 3. Recent scarlet fever • *A firm diagnosis requires 1) 2 major manifestations OR 1 major and 2 minor manifestations and 2) evidence of a recent streptococcal infection. • However, when chorea or carditis is clearly present, evidence of an antecedent group A streptococcal infection is not necessary.

  9. Migratory polyarthritis • This is usually an early feature and most common • acute, painful, asymmetric and migratory inflammation of the large joints (typically the knees, ankles, elbows and wrists). • The joints are involved in quick succession and are usually red, swollen and tender for between a day and up to 4 weeks. • The pain characteristically responds to aspirin; if it does not, the diagnosis is in doubt.

  10. 2. Carditis : • All 3 layers can be involved ( Pancarditis) • Cardiomegaly, congestive heart failure   Acute pericarditis, pericardial effusion  • Apical pansystolic murmur (mitral regurgitation) • Apical mid-diastolic murmur (Carey Coombs) • Basal diastolic (aortic regurgitation)

  11. 3. Erythema marginatum occurs in less than 5% of patients. The lesions start as red macules (blotches) which fade in the center but remain red at the edges and occur mainly on the trunk and proximal extremities • Often associated with chronic carditis

  12. 4. Subcutaneous nodules occur in 5-7% of patients. They are small (0.5-2.0 cm), firm and painless, and are best felt over extensor surfaces of bone or tendons. • Always associated with severe carditis

  13. 5. Sydenham's chorea (St Vitus dance) • Involuntary choreiform movements of the hands, feet or face. Speech may be explosive and halting. Spontaneous recovery usually occurs within a few months. • Clinical signs- pronator sign, jack in the box sign , milking sign of hands

  14. INVESTIGATIONS IN ACUTE RHEUMATIC FEVER • Evidence of a systemic illness (non-specific) • Leucocytosis, raised ESR, raised CRP • Evidence of preceding streptococcal infection (specific) • Throat swab culture: group A ß-haemolytic streptococci (also from family members and contacts) • Antistreptolysin 0 antibodies (ASO titres): rising titres, or levels of > 200 U (adults) or > 300 U (children) • Evidence of carditis • Chest X-ray: cardiomegaly; pulmonary congestion • ECG: first- and rarely second-degree heart block; features of pericarditis; T-wave inversion; reduction in QRS voltages • Echocardiography: cardiac dilatation and valve abnormalities

  15. Treatment of the acute attack • Bed rest and supportive therapy • The duration of bed rest should be guided by symptoms and markers of inflammation (e.g. temperature, leukocyte count and ESR and should be continued until these have settled. • Treatment of congestive cardiac failure: digitalis, diuretics,ACE inhibitor • Treatment of chorea: diazepam or haloperidol • Rest to joints & supportive splinting

  16. Pain relief • Aspirin • A reasonable starting dose is 60 mg/kg body weight per day, divided into six doses. • In adults, 100 mg/kg per day may be needed up to the limits of tolerance or a maximum of 8 g per day. • Aspirin should be continued until the ESR has fallen and then gradually tailed off • Corticosteroids • These produce more rapid symptomatic relief than aspirin, and are indicated in cases with carditis or severe arthritis. • Prednisolone, 1.0-2.0 mg/kg per day in divided doses, should be continued until the ESR is normal then tailed off.

  17. Antibiotic • A single dose of Benzathine penicillin 1.2 million U i.m.(after skin sensitivity) or oral phenoxymethylpenicillin 250 mg for children and 500mg for 6-8hourly for 10 days • If the patient is penicillin-allergic; erythromycin

  18. PREVENTION IN RF • Preventing Strep Throat - Vaccine ? • Treating Strep Throat infection • Preventing Rheumatic recurrence by chemoprophylaxis • Treating RHD

  19. SECONDARY PROPHYLAXIS • Continuous chemoprophylaxis to prevent recurrence in a patient who had an initial attack of RF • STRATEGY • Chemoprophylaxis • Treat breakthrough Infection

  20. Chemoprophylaxis •  BPG 1.2 million U IM 3 weekly • Penicillin V 250 mg BD PO daily • Erythromycin 250 mg BD PO daily • Sulfadiazine 500 mg OD PO daily 1000 mg

  21. Secondary rheumatic fever prophylaxis • Bezathine penicillin 1.2 million unit i.m. – every 3-4 weeks

  22. CHRONIC RHEUMATIC HEART DISEASE

  23. Rheumatic heart disease (RHD) is the long-term consequence of acute rheumatic fever during childhood. • As the initial damage (inflammation) subsides, scar tissue forms leaving the valves either too narrow (stenotic) or too "leaky" (insufficient); also atrial dilation, arrhythmias, and ventricular dysfunction • Rheumatic heart disease refers to this permanent scarring of the heart valves

  24. Two-thirds of cases occur in women • The mitral valve is affected in more than 90% of cases; the aortic valve is the next most frequently affected, followed by the tricuspid and then the pulmonary valve • Isolated mitral stenosis accounts for about 25% of all cases • develops 2-10 years after an episode of acute rheumatic fever, and recurrent episodes may cause progressive damage to the valves.

  25. Treatment: • Monthly treatment with benzathine penicilline IM to prevent recurrences • Afterload reduction (ie, using ACE inhibitor captopril) for heart failure • For symptomatic congestive heart failure uncontrolled by medication, surgery may involve repairing a damaged valves without replacing it • Permanent pacemaker may be required for AF

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