rheumatic heart disease BY BUSHANGALA MARK.pptx

GOOD REVISION NOTES ON RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE 2024

1. Rheumatic Fever and rheumatic heart disease Presented by group 1, compiled by bushangala mark

2. DEFINITION: Rheumatic fever is a non- suppurative , acute inflammatory complication of group A streptococcal infection, causing combinations of arthritis, carditis, subcutaneous nodules, erythema marginatum , and chorea. It is a diffuse inflammatory disease of connective tissue primarily involving heart, blood vessels, joints, sub-acute tissue and CNS 2

3. Etiology Beta hemolytic streptococci. Genetic certain genetic factors may increase an individuals susceptibility to autoimmune reactions 3

4. Epidemiology Ages 5-15 yrs are most susceptible Rare <3 yrs Girls>boys Environmental factors-- over crowding, poor sanitation, poverty , (these factors favor transmission or poor recognition and early management of strep. infections 4

5. Pathogenesis Delayed immune response to infection with group A beta hemolytic streptococci. Rheumatic fever is an inflammatory disease that occurs as a complication of streptococcal pharyngitis, caused by Group A Streptococcus (GAS) bacteria. The pathogenesis of rheumatic fever involves a complex interplay between the host immune response and the infectious agent. 1. Initiating Infection: Group A Streptococcal Pharyngitis Rheumatic fever typically follows a throat infection caused by Group A Streptococcus (GAS), specifically Streptococcus pyogenes . 5

6. Continued… 2. Molecular Mimicry and Cross-Reactivity The primary mechanism in the pathogenesis of rheumatic fever is molecular mimicry . The streptococcal bacteria have cell wall components, such as the M protein , that are structurally similar to certain proteins found in human tissues (e.g., in the heart, joints, skin, and central nervous system). When the immune system responds to the GAS infection, it produces antibodies and T-cells targeted against the M protein. Due to the structural similarity, these antibodies and T-cells also cross-react with human tissues, particularly affecting the heart, joints, brain, and skin. 6

7. Continued… 3. Inflammatory Response and Tissue Damage The autoimmune response leads to inflammation and tissue damage, which is characteristic of rheumatic fever: Heart (Carditis): The most serious manifestation of rheumatic fever is rheumatic carditis, which can affect all layers of the heart (pancarditis). Endocarditis: Affects the inner lining of the heart and most commonly involves the heart valves, particularly the mitral and aortic valves, leading to valvulitis . Myocarditis: Inflammation of the heart muscle itself. Pericarditis: Inflammation of the outer layer of the heart. The damage to the heart valves can result in chronic rheumatic heart disease (RHD), characterized by valvular stenosis and/or regurgitation.

8. continued Joints (Migratory Polyarthritis): Inflammation commonly affects the large joints such as knees, ankles, elbows, and wrists. The arthritis is typically migratory, meaning it moves from one joint to another. Central Nervous System (Sydenham's Chorea): This condition, also known as St. Vitus' dance, involves involuntary, rapid, and irregular movements. It is thought to result from autoimmune reactions targeting neurons in the basal ganglia. Skin (Erythema Marginatum and Subcutaneous Nodules): Skin manifestations include a characteristic rash known as erythema marginatum and the presence of subcutaneous nodules. 8

9. Pathologic Lesions - Pan- carditis in the heart - Arthritis in the joints - Ashcoff nodules in the subcutaneous tissue, heart -Basal gangliar lesions resulting in chorea 9

10. Clinical Features Migratory polyarthritis, involving major joints Commonly involved joints-knee, ankle, elbow & wrist Involved joints are exquisitely tender In children below 5 years arthritis usually mild but carditis more prominent Arthritis do not progress to chronic disease 10 1.Arthritis

11. Clinical Features (Cont’d) 11 Mainly in girls of 1-15 yrs age May appear even 6 months after the attack of rheumatic fever Clinically manifest as-clumsiness, deterioration of handwriting, emotional lability or grimacing of face, abnormal posturing Clinical signs- pronator sign . 2.Sydenham Chorea

12. Clinical Features (Contd) Unique, transient, skin lesions of 1-2 inches in size Pale center with red irregular margin More on trunks & limbs & non-itchy Worsens with application of heat 12 3.Erythema Marginatum

13. Clinical Features (Contd) Painless, pea-sized, palpable nodules Mainly over extensor surfaces of joints, spine, scapulae & scalp. 13 4.Subcutaneous nodules

14. Clinical Features (Contd) Fever Arthralgia Pallor Anorexia Loss of weight 14 Other features (Minor features)

15. Manifest as pancarditis (endocarditis/ valvulitis , myocarditis and pericarditis). Carditis is the only manifestation of rheumatic fever that leaves a sequel & permanent damage to the organ. Chronic phase-fibrosis, calcification & stenosis of heart valves . 16 5.Carditis (RHEUMATIC HEART DISEASE )

16. PERICARDITIS Present as a sharp stabbing chest pain that gets worse when pt. in supine, lie flat, cough, swallow and is relieved on leaning forward or sits forward Fever, anxiety, pain in the back, neck and shoulder, Ewart’s sign: dullness to percussion, bronchial breath sounds appreciated at inferior angle of left scapula in case of pericardial effusion compressing the left lower lobe of lung.

17. Pericarditis continued… Beck’s triad(associated with cardiac tamponade ) Palpitations Dyspnea due to constriction or cardiac tamponade . Jugular venous distension Kussmaul’s sign (paradoxical rise in JVP during inspiration contrary to usual decrease in this phase

18. ENDOCARDITIS Inflammation of the inner lining of the heart , the endocardium, usually involving the valves and other structures like the intra-ventricular septum Valvular damage may include mitral stenosis, mitral regurgitation, aortic stenosis, aortic regurgitation

19. MYOCARDITIS…( discuss)

20. Laboratory Findings High ESR Anemia, leukocytosis (CBC) Elevated C-reactive protein ASO titer, increased (Peak value attained at 3 weeks, then comes down to normal by 6 weeks) Throat swab culture- for beta hemolytic streptococci 21

21. Laboratory Findings (Contd) ECG- prolonged PR interval ,ST depression, T inversion 2D Echo cardiography: valve edema, mitral regurgitation, LA & LV dilatation, and decreased contractility 22

22. 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 23

23. 24 Evidence of recent streptococcal infection – Elevated ASO-titer or other streptococcal Ab. titers or positive throat swab for group A beta-hemolytic streptococcus PLUS Two major manifestations or one major and two minor manifestations

24. Differential diagnoses Infective endocarditis SLE Juvenile idiopathic arthritis Viral myocarditis Reactive arthritis

25. 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) 26

26. 27

27. 28

28. Prognosis Rheumatic fever can recur whenever the individual experience new Beta hemoltic 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 valvular lesions 29

29. prevention Early diagnosis and treatment of group A Streptococcus throat infection Avoid overcrowding, good housing Good nutrition

30. THANK YOU 31