acute rheumatic fever in pediatrics .pptx

Rheumatic fever is very important for pediatrics patients

1. Acute rheumatic fever

2. Introduction  it is multisystem autoimmune response to sore throat caused by Group A beta hemolytic streptococci (GAS infection). Occurs to molecular mimicry Latency 2-3 weeks (after sore throat) 100% of all acute rheumatic fever are due to GAS infection

3. Streptococcal sore throat ( rheumatogenic strains)  cause Rheumatic fever Streptococcal pyoderma ( Nephrogenic strain)  causes PSGN

4. Progression of untreated disease GAS infection Acute rheumatic fever Rheumatic heart disease (d/t recurrent rheumatic fever) Heart failure

5. After GAS infection 0.3 – 3 % develops acute rheumatic fever 0.3 % seen in non-epidemic area 3% seen in epidemic area 30% of all sore throat in childrens are GAS related (streptococcal nature)

6. Triad Susceptible host Agent  GABHS environment

7. AGENT GAS  (a) Group A carbohydrate  stimulates B-cells and produce antibodies against Group A carbohydrate (which cross react with N-acetyl-Beta-D-Glucosamine, Laminin , Laminar basement membrane) (b) M protein T cell stimulation against M protein (which is cross react with cardiac myosin)

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9. Host 3-6% lifetime cumulative incidence of RF in population exposed to rheumatogenic GAS infection regardless of geography/ethnicity. Familial aggregation  5 times risk Monozygotic twins 6 times than dizygotic twins Heritability of RF is 60% HLA-DR 4 and HLA-DR 7 are susceptible

10. Environment A disease of social justice  poverty, overcrowding, malnutrition, illiteracy, poor health care and poor sanitation. 97% of all new rheumatic fever cases occur in developing countries.

11. RHD develops 5-15 years after Acute RF .

12. Pathogenesis

13. Clinical features Seen in 5-15 years old children ARF  affects males and females eaqually RHD  more common in females All the manifestations of Acute RF affects equally males and females except sydhenams chorea

14. Arthritis  most common Carditis  2 nd most common Chorea Subcutaneous nodule Erythema marginatumrare

15. Rare clinical manifestations of rheumatic fever Epistaxis Abdominal pain Rheumatic pneumonia and pleurisy

16. Younger the patient more chance of developing  CARDITIS Older the patient more chance of developing  ARTHRITIS

17. Arthritis Asymmetric migratory polyarthritis Predominantly lower limbs  knee > ankle> wrist > hip> elbow> shoulder Severe Pain and tenderness out of proportion to erythema , warmth and swelling Usually resolves within 4 weeks No residual deformity

18. Synovial fluid  sterile inflammatory fluid Decreased complements  C1q, C3, C4 X-ray s/o joint effusion (but no any joint erosions) Dramatic response to NSAIDS

19. Jaccouds arthritis / arthropathy d/t recurrent rheumatic fever involve fibous articular capsule No joint erosion Involve small joints Painless No any inflammatory markers increased

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21. Carditis Rheumatic fever is a Pancarditis . It is long term clinical sequelae Pericarditis and heart failure never occur in absence of valve lesion Causes mitral valvulitis Rheumatic carditis basically endocarditis

22. Clinical criteria – No echo -- Endocarditis -- Myocarditis -- Pericarditis -- pansystolic murmur mitral reguritation , carey-coombs murmur, end diastolic murmur  aortic regurgitation Cardiomegaly Heart failure S3 gallop Heart block (commonly 1 st degree block) -- Rub effusion

23. 1944 to till 2015  diagnose carditis clinically  we needed to have tachycardia with murmurs but having problems interobserver variation, severe MR (severe tachycardia), obese, or in HF and shock Since 2015  we utilise Echocardiography

24. Clinical carditis With a valvulitis murmur. As fullfilling the major criteria of carditis in all population.

25. Subclinical carditis A major change in 2015 revision of the jones criteria is the acceptance of subclinical carditis . Echo for all patients with rheumatic fever Echo to be serially repeated 80% of patients who develop carditis do so in the first two of onset of rheumatic fever If in suspected case we repeat the echo within 1 st 14 days.

26. Criteria to differentiate pathologic from physiologic murmur Color jet of length >1 cm in AR and >2 cm in MR. Jet seen in at least 2 planes. Peak velocity >3 m/s. Doppler signal holosystolic for MR and holodiastolic for MR

27. Warty apperance of mitral valve it is characteristic of rheumatic fever Small vegetation adherent along with line of closure valve. and never embolise

28. Myocarditis Does not satisfy Dallas criteria HF is solely due to acute regurgitation No cardiac enzymes rise

29. Pericarditis Fibrinous Signifies active carditis Bread and butter pericarditis

30. Sydenahms chorea Due to immune mediated reaction to autoantibodies of basal ganglia ( increased dopamine secretion) It occurs late May be only presenting manifestation  15% cases. Females  most common Mostly in young females childrens Never seen in postpubertal males Latent period 6-8 weeks chorea 8-15 weeks (1-2 years) after resolves Disappears during sleep

31. Jack in the box tongue / trombone tongue Tongue fasciculations Milkmaid grip Hyperpronation of arm Facial tics Emotional liability Can become patient socially distressing Inflammatory markers may be normal

32. Subcutaneous nodule Firm, painless, freely mobile over subcutaneous tissue Present over the occiput , elbows. Knees, ankle and tendo-achilles Occur in 1.5 % of patients Also seen in prolonged carditis Multiple crops indicates severe carditis

33. Erythema marginatum Rarest menifestation transient pink rash with central clearing non- pruritic in nature Provoked by hot bath Commonly seen over trunk and upper limbs but not over the face Indicates milder forms of carditis

34. Syndromic diagnosis No single symptom or sign or lab test is diagnostic of ARF. Jones criteria provides weightage to individual clinical feature major or minor to make a diagnosis. Criteria are based on specificity not frequency.

35. Step 1 – establish population at risk Step 2 – establish preceding GAS infection Step 3 – apply major and minor criteria

36. Population at risk Low risk population  -- those with ARF incidence </= 2 per lakh school age children or -- all age RHD prevalence of </=1 per 1000 population per year Moderate-High risk population -- those with ARF incidence > 2 per lakh school age children or -- all age RHD prevalence of >1 per 1000 population per year

37. Establish preceding GAS infection Evidence of preceding GAS infection -- throat swab culture  positive in 11% cases. -- positive rapid group A carbohydrate antigen test -- rising titre of ASLO / DNAse -B ( cutt of value ASLO - 333 todd units for childrens and 250 todd units for adults) ( cutt of value Anti DNAse – 240 todd units for childrens and 120 todd units for adults)

38. Major and minor criteria

39. Need not look at criteria Sydenahms chohrea  acute RF Rheumatic MS

40. TREATMENT  suppresive therapy Only suppress the symptoms Not modify the disease Prevent recurrence Do not decrease the incidence of rheumatic heart disease Does not alter duration of illness

41. Goals of treatment Suppression of inflammatory response minimise effects of inflamation on hearts and joints Eradication of GAS from pharynx Symptomatic relief Commence secondary prophylaxis Treatment of HF Bed rest IM benzathine penicillin to eradicate any residual GAS infection

42. Anti-inflammatory agents Aspirin and other NSAIDS (naproxen) Steroids Arthritis  aspirin /NSAIDS superior

43. Salicylates vs corticosteroids in carditis Mild carditis – aspirin / NSAIDS Moderate and severe carditis – start with steroids while tapering steroids starts aspirin.

44. Prophylaxis Aim  prevent recurrence of rheumatic fever Primordial prevention prevents the GAS infection  via improve social factors and give vaccine. Primary preventionprevents the 1 st episode of acute rheumatic fever treat the sore throat with oral penicillin for 10 days or single inj. Of benzathine penicillin. Secondary prevention  prevents the recurrence of rheumatic fever Tertiary preventionprevents the heart failure

45. Primary prevention DOC  benzathine penicllin ; single IM 1.2 miilion units, or weight <27 kg give ½ of dose. Phenoxymethylpenicillin (penicillin V): oral for 10 days 250-500 mg three times a day. Other alternatives  erythromycin, azithromycin , clarithromycin , and clindamycin .

46. Secondary prevention Regular delivery of benzathine penicillin Secondary prophylaxis  -- reduces severity of RHD -- helps prevent deaths from RHD  Incidence of rheumatic fever recurrence -- 20% in first 5 years -- 10% in next 5 years -- 5% in next 5 years

47. Benzathillin penicillin  single IM evry 3-4 weeks Penicillin V  Oral 250 mg bd Sulfonamides  oral >/= 30 kg 1 gm od Erythromycin  oral 250 mg bd

48. Duration of prophylaxis – WHO guidelines - Category - Duration 1) Rheumatic fever without carditis -- 5 years or 21 years of age , whichever is longer 2) Rheumatic fever with carditis but without residual heart disease ( no valvular disease) -- 10 years or until 21 years of age , whichever is longer 3) Rheumatic fever with carditis and residual heart disease (persistent valvular disease) -- 10 years or until 40 years of age, whichever is longer , sometimes lifelong prophylaxis

49. Tertiary prevention Prevention of heart failure Ballon valvuplasty Heart valve replacement Capacity building for treatment

50. Thankyou