acute Rheumatic fever it's management.pptx

Rheumatic fever

1. Acute Rheumatic Fever Dr S V Ramanamurty MD, FIACM, FICP, FACP (USA)

2. Rheumatic Fever Definition A multisystem inflammatory disorder occurring as a delayed sequel to pharyngeal infection with Group A Beta Hemolytic streptococci It primarily involves heart, joints, CNS, skin & subcutaneous tissues

3. Epidemiology Rheumatic fever is world wide It is a major cause of death and disability in children & adolescents in socioeconomically deprived areas Over-crowding and substandard housing predispose to rheumatic fever

4. Epidemiology Age of incidence : 5-15 years Males and females are equally affected People with HLA DR-3 are more prone to develop

5. Pathogenesis Molecular mimicry appears to play an important role in the pathogenesis of Rheumatic Fever Rheumatic fever occurs as an immunological sequel Latent period : 2-15 weeks after streptococcal sore throat Streptococcus induced autoimmunity is believed to be the mechanism in rheumatic process

6. Pathogenesis Several streptococcal antigens have been demonstrated to have cross reactivity with cardiac and other tissues ( Molecular mimicry ) Super antigens There are shared epitopes between streptococcal M protein and cardiac myosin This leads to cross-reactivity between human heart & streptococci

7. Pathology General Acute rheumatic fever is characterized by Exudative & Proliferative lesions --------------------------------------------------------------------- Exudative lesions are seen in acute lesions (in joints) They heal completely Proliferative lesions are seen in prolonged & chronic lesions usually in heart valves

8. Aschoff Body Hallmark lesion of rheumatic fever Characterized by localized Areas of Fibrinoid degeneration Surrounded by Plasma cells Lymphocytes, Mononuclear and Basophilic giant cells

9. Pathology ARF involves heart, joints, CNS, skin, and subcutaneous tissues Carditis : all three layers are involved Myocarditis, pericarditis & endocarditis Myocarditis : Fragmentation of fibroblasts Infiltration of lymphocytes Presence of Aschoff bodies

10. Pathology Pericarditis Characterized by deposition of sero-fibrinous exudates giving a naked eye appearance of Bread and butter appearance Occasionally pericardial effusion Endocarditis Characterized by verrucous valvulitis, formation of small rheumatic nodules, on the atrial surface of the mitral valve The mitral valve is most commonly affected, next aortic, & tricuspid valves very rarely pulmonary valve

11. Pathology Joints : involvement with effusion (Poly Arthritis) Usually heal without residual lesions Subcutaneous nodules Show histological features of Aschoff bodies Pleural effusion: Fibrinous pleurisy Pulmonary lesions : Rheumatic pneumonitis Brain parenchyma : Nonspecific lesions

12. Clinical features General After a latent period of 1-5 weeks of sore throat Fever Anorrhexia Arthralgia Palpitations Lethargy Night sweats

13. Clinical features of rheumatic fever

14. Clinical features Major features Carditis Arthritis Subcutaneous nodules Erythema marginatum Chorea

15. Clinical features Carditis occurs earlier within 3 weeks of sore throat It includes Myocarditis, pericarditis, endocarditis Myocarditis Tachycardia disproportionate to fever Dropped beats Tic-tac rhythm / fetal rhythm Arrhythmias – prolonged PR interval S 3, S 4, or Summation gallop Congestive heart failure

16. Clinical features Pericarditis Pericardial friction rub, pericardial effusion with increased area of dullness, Ewart sign (bronchial breathing near inferior angle of left scapula Endocarditis Mitral area: MDM (carey-combs murmur) due to rheumatic nodules Pan-systolic murmur due to Mitral Regurgitation Aortic area Early Diastolic Murmur due to Aortic Regurgitation

17. Areas of cardiac auscultation

18. Clinical features Note Carditis to be considered with A combination of Cardiomegaly Pericarditis Congestive heart failure

19. Clinical features Polyarthritis It is the most common manifestation of ARF 75 %, usually in 4-6 weeks of sore throat Large joints, asymmetrically involved with fleeting Joint pains (two or more joints involved) Red, tender, swollen Migratory in nature spine rarely involved Heal completely, without residual lesions

20. Clinical features Subcutaneous nodules Seen in 10% with severe Carditis Nodules are small, pea sized, painless & Movable, Present on dorsal aspect of knees, ankles, elbows & scalp Would be present for about 2-3 weeks

21. Clinical features Erythema marginatum : present in about 10% cases Erythmatous macules, with red rounded or serpiginous margins and clear centers They are migratory, transient and evanescent, non- pruritic , non- indurated Blanch with pressure and brought back with application of heat Present usually over trunk or proximal parts of limbs

22. Erythema marginatum :

23. Chorea Develops usually late around 6-9 months after the initial sore throat Usually seen in Female children 7-14 yrs. Obsessions and compulsions common Defined as sudden, jerky, pleomorphic, non-repetitive, quasi-purposive, involuntary movements

24. Clinical features Other features Fever Arthralgia Epistaxis Pain abdomen

25. Duration of the attack The average duration of an untreated rheumatic fever is around 3 months If it exceeds 6 months, it is called Chronic rheumatic fever

26. Investigations Isolation of Group A Beta hemolytic streptococci By Culture of throat swab (only in a minority of cases) Streptococcal Antibody Tests (Serological tests) These tests confirm recent streptococcal infection Anti- streptolysin O (ASO test) Anti – D Nase B test Anti Hyaluronidase (AH test) Anti Streptozyme (ASTZ test)

27. Investigations Serological tests Single titers of ASO >250 todd units in adults & 333 todd units in children >5years taken as positive A rising titer is more significant ------------------------------------------------------------------------- Anti Streptozyme (ASTZ test) is a very sensitive indicator of recent streptococcal infection. Titers >250 units/ml are considered positive If it is negative it rules out streptococcal infection

28. Investigations Acute phase reactants These tests indicate presence of an inflammatory process ESR raised C-reactive protein is raised Note These two are normal in chorea

29. Investigations Other tests confirming an inflammatory reaction Polymorphonuclear leucocytosis Increase in serum complements Increase in serum mucoproteins , alpha 2 & gamma globulins Anemia due to suppression of erythropoiesis

30. Investigations ECG –may show prolonged PR interval Chest X Ray PA view – Cardiomegaly, pulmonary congestion Echocardiography can detect Myocardial dysfunction Valve dysfunction Pericardial effusion

31. ECG & X-Ray chest Prolonged PR interval Cardiomegaly Pulmonary congestion

32. Complications Arrhythmias Congestive heart failure Rheumatic heart disease Pericarditis Myocarditis

33. Differential diagnosis Infective endocarditis Rheumatoid arthritis Sickle cell anemia SLE Osteomyelitis Chronic meningococcemia

34. Diagnosis of acute rheumatic fever Major criteria Carditis Polyarthritis Chorea Erythema marginatum Subcutaneous nodules Minor criteria Fever Arthralgia Previous rheumatic fever Raised ESR Positive CRP Prolonged PR- interval (ECG) Revised Jones criteria 1992

35. Diagnosis of acute rheumatic fever Essential criteria: Evidence of preceding streptococcal infection Recent scarlet fever or Positive throat culture for group A Streptococci or Streptococcal antibodies in high titers Confirmed diagnosis ---------------------------------------------------------------------------------------------- [ Two major or One major + two minor ] + { one essential criteria }

36. Revised Jones criteria 2015 Low risk population : Cases of acute rheumatic fever ≤ 2per 1,00,000 population school-age children or prevalence of chronic rheumatic carditis in any age group lower than or equal to 1/1000 per year. High risk population Children from communities that exhibit levels above these would have moderate-to-high risk for acquiring the disease.

37. Revised Jones criteria 2015 Low risk population High risk population Carditis Arthritis – only by polyarthritis Chorea Erythema marginatum Subcutaneous nodules Carditis (clinical or by Echo Arthritis – monoarthritis or Poly. Polyarthralgia Chorea Erythema marginatum Subcutaneous nodules Major criteria

38. Revised Jones criteria 2015 Low risk population High risk population Minor criteria Polyarthralgia Fever ≥ 38.5 C ESR ≥60 mm/ hour, CRP 3.0mg/ dL Prolonged PR interval (if there is no carditis as major criteria) Monoarthralgia Fever ≥ 38 C ESR ≥30 mm/ hour, CRP 3.0mg/ dL Prolonged PR interval (if thereis no carditis as major criteria )

39. Management Bed rest For patients without Carditis – rest until Temperature & ESR become normal For patients who developed Carditis – rest for 2-6 weeks after the ESR & temperature have returned to normal

40. Management Anti – streptococcal therapy Single inj. of benzathine penicillin 1.2 million units IM (or) Inj. Procaine Penicillin 6 lacks im daily for 10 days -------------------------------------------------------------------------- For those who are allergic or sensitive to penicillin Oral erythromycin 20-40mg /kg /day in three divided doses for 5 days (or) Oral Azithromycin 500mg/day for five days

41. Management Salicylates Aspirin is useful for symptomatic relief For children: Started at 60mg/kg/ day in six divided doses. For adults 100mg/kg , increased gradually up to 8 grams per day until the drug produces side effects or clinical improvement Aspirin should be continued at this dose until ESR comes to normal, Then dose is gradually tapered Side effects Mild toxicity : Nausea, tinitus , deafness, vomiting, Severe: Tachypnoea, acidosis

42. Management Corticosteroids Indications Patients who have severe Carditis, manifested by heart failure not responding to aspirin Patients with severe arthritis not responding to aspirin Prednsolone is given at a dose of 60-120 mg /day in four divided doses until ESR is normal It is then tapered off gradually over a period of 4-6 weeks

43. Management Supportive therapy Treatment of Heart failure Heart blocks Chorea

44. Prevention of rheumatic fever Primary prevention – Secondary prevention-

45. Prevention of rheumatic fever Primary prevention – Accurate diagnosis and treatment of pharyngitis with Group A Beta Hemolytic streptococci with inj Procaine Penicillin 6 lacks IM daily for 5 days Sore throat in children in close communities where rheumatic fever is endemic, all children 5-12 years to be treated for sore throat

46. Prevention of rheumatic fever Secondary prevention Rheumatic fever prophylaxis to be offered to all patients who have documented diagnosis of rheumatic fever Duration of prophylaxis If No carditis – for 5 years or until 18 years of age If Resolved carditis – for 10 years or until 25 years of age If Severe RHD – offered for 40years or lifelong

47. Regimen offered Benzathine penicillin 12lacks Units IM once in 3 weeks (or) Oral penicillin V 500mg twice a day ----------------------------------------------------------------- Erythromycin 250mg twice a day orally Azithromycin 500mg once day (for patients who are allergic to penicillin)

48. What next

49. Thank you