Rheumatic fever clinical features and diagnosis

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rheumatic fever, aetiopathogenesis, clinical features and diagnosis

rheumatic fever, aetiopathogenesis, clinical features and diagnosis

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  • 1. RHEUMATIC FEVERClinical features and diagnosis DR . SUJIT SAHU http://cardiologysearch.blogspot.in/
  • 2.  INTRODUCTION HISTORICAL BACKROUND EPIDEMIOLOGY PATHOGENESIS PATHOLOGY CLINICAL FEATURES DIAGNOSIS http://cardiologysearch.blogspot.in/
  • 3. INTRODUCTION Clinical syndrome Acute , non-suppurative inflammatory disease following Group A Beta Hemolytic Streptococcal sore throat Classified as Connective tissue disease or collagen vascular disease affecting the Joints, heart , brain , skin and subcutaneous tissue http://cardiologysearch.blogspot.in/
  • 4. HISTORICAL BACKROUND 1604 – Guilleaume (France) Thomas Syndenham (Eng) -Polyarthritis 1605 - Sydenham - St. Vitus Dance 1761 - Morgani (Italy) – Heart valves 1813 - W.C.wells – Subcutaneous Nodules 1818 - Laennec - RHD (clinical) http://cardiologysearch.blogspot.in/
  • 5. HISTORICAL BACKROUND 1886 - Cheadle - Full syndrome 1904 - Aschoff - Aschoff Nodule 1931 - Coburn - Streptococcal assoc. 1944 - Jones - Criteria 1951 - Wannamaker (penicillin prophylaxis) http://cardiologysearch.blogspot.in/
  • 6. EPIDEMIOLOGYhttp://cardiologysearch.blogspot.in/
  • 7. EPIDEMIOLOGYhttp://cardiologysearch.blogspot.in/
  • 8. India S Padmavati Director, National Heart Institute, New Delhi, India In 2000, in a school survey involving 3963 children from the district of Kanpur, the prevalence of RHD was 4.54 per 1000 (Urban 2.56 and Rural 7.42). The prevalence of RF was 0.75 per 1000 (Rural 1.20, Urban 0.42) http://cardiologysearch.blogspot.in/
  • 9. EPIDEMIOLOGY 2000 - 2004HOSPITAL BASED SURVEYS : Agarwal et al (varanasi) : Decreasing (8.4% - RHD & 1.1% RF) Despande et al (Mumbai): No change Mishra et al (cuttack) : No change http://cardiologysearch.blogspot.in/
  • 10. EPIDEMIOLOGYPREVELANCE : 2 million at presentINCIDENCE : 50 000 new cases every year http://cardiologysearch.blogspot.in/
  • 11. PATHOGENESI S http://cardiologysearch.blogspot.in/
  • 12. STRUCTURE OF Group –ABeta Hemolytic Streptococcus http://cardiologysearch.blogspot.in/
  • 13. Group - A Streptococcus Two highly conserved epitopes within M protein divide GAS immunologically into Class I (throat) Class II (skin) strains. All RF strains fall clearly into Class I throat strains The site of infection must be pharyngeal . Regardless of how virulent an invasive strain may be, ARF does not result when it is introduced extra- pharyngeally, e.g. through skin lesions or wound infections http://cardiologysearch.blogspot.in/
  • 14. CROSS REACTIVITY http://cardiologysearch.blogspot.in/
  • 15. CO-PATHOGENS Burch et al & Pongpanich et al : (1970) (1976) Serological evidence of Cox B viruses in patients with rheumatic fever http://cardiologysearch.blogspot.in/
  • 16. GENETIC PREDISPOSITION Specific B - cell alloantigen HLA DR 3 - Indians Moari races in New Zealand & Samoans in Hawaii High concordance in twins Increased risk in families with H/O RF http://cardiologysearch.blogspot.in/
  • 17. ENVIRONMENT Low socio-economic group Urban slums Poor accesibility to health care Over crowding Unclean environmentMostly seen in developing countries http://cardiologysearch.blogspot.in/
  • 18. INTERACTIONhttp://cardiologysearch.blogspot.in/
  • 19. http://cardiologysearch.blogspot.in/
  • 20. PATHOLOGYhttp://cardiologysearch.blogspot.in/
  • 21. INFLAMMATORY RESPONSE Edematous change Cellular infiltrate Fibrinoid necrosis Aschoff body (seen only in heart)http://cardiologysearch.blogspot.in/
  • 22.  Joints : serositis Pericardium Skin (S/C nodule) : Fibrinoid Heart degeneration Erythema Marginatum : Vasculitis Chorea : Vasculitis http://cardiologysearch.blogspot.in/
  • 23. ASCHOFF BODY  Granuloma  Central fibrinoid necrosis  Surrounded by lymphocytes, Antischkow cells and Plasma cellshttp://cardiologysearch.blogspot.in/
  • 24. http://cardiologysearch.blogspot.in/
  • 25. http://cardiologysearch.blogspot.in/
  • 26. http://cardiologysearch.blogspot.in/
  • 27. http://cardiologysearch.blogspot.in/
  • 28. Initial edemaHyaline degenerationVerrucae formation at the edge of leafletsPrevents approximation RegurgitationFibrosis & calcification Stenosis
  • 29. http://cardiologysearch.blogspot.in/
  • 30. ORDER OF VALVE INVOLVEMENT Mitral Aortic Tricuspid Pulmonary http://cardiologysearch.blogspot.in/
  • 31. INTERNATIONAL SERIES BY BONOW  PURE MS : 25 %  PURE MR : 10 %  MS / MR : 25 %  AORTIC : 8%  ALL VALVES : 7% http://cardiologysearch.blogspot.in/
  • 32.  CLINICAL FEATURES http://cardiologysearch.blogspot.in/
  • 33. PERCENTAGE INVLOVEMENT (Indian Scenario) ARTHRITIS : 70 % ARTHALGIA : 90 % CARDITIS : 70 % CHOREA : 08 % S/C NODULE : 02 % ERYHTEMA MARGINATUM : 01 http://cardiologysearch.blogspot.in/
  • 34. LATENCY From onset of sore throat to onset of initial attack of rheumatic fever is 1 – 5 weeks for recurrent attacks Median of 19 days & shorter http://cardiologysearch.blogspot.in/
  • 35. LATENCY Joint manifestations are first to occur - heralding onset of disease Carditis occurs within 2 weeks - is apparent when patient is first seen Subcutaneous nodules appear 4 weeks or more after onset of symptoms Chorea may appear 2 to 6 months later Erythema marginatum occurs both early & later http://cardiologysearch.blogspot.in/
  • 36. MODE OF ONSET Variable Abrupt onset with fever & acute polyarthritis Insidious or sub clinical in mild indolent carditis May present with CCF May present atypically with acute abdomen due to peritoneal inflammation http://cardiologysearch.blogspot.in/
  • 37. POLYARTHRITIS Most common & Least specific severe in adults Large joints ; asymetrical Flitting- involves joints after joints Fleeting - Lasting for short time 3 days - 1 week No residual damage http://cardiologysearch.blogspot.in/
  • 38. POLYARTHRITIS Responds dramatically to aspirin Severity inversely related to carditis (Feinstein & Spagnuola et al – 1962) JACCOUDS ARTHRITIS :  Smalljoints  Produces residual damage  Seems to be related to RF http://cardiologysearch.blogspot.in/
  • 39. PANCARDITIS More severe in the young Sub clinical to fulminant ENDOCARDITIS :  AR : 20 %  MR : 75 % : due to - Valvulitis - MVP (anterior leaflet) - Annular dysfunction http://cardiologysearch.blogspot.in/
  • 40. ENDOCARDITISClinical Evidence of Endocaritis :  Apical holosystolic murmur  Carey coomb’s murmur  Early diastolic murmur http://cardiologysearch.blogspot.in/
  • 41. MYOCARDITIS Clinical evidence of Myocarditis : Cardiomegaly Clinical features of CHF Gallop rhythm http://cardiologysearch.blogspot.in/
  • 42. PERICARDITIS Clinical evidence of Pericarditis : Pericardial rub Associated with endocarditis Indicates severe carditis (High rheumatic activity) No residual constriction http://cardiologysearch.blogspot.in/
  • 43. CHOREA Occurs 3 months later than other RF features - spontaneous resolution Duration : variable ( upto 6 months) Often in prepuberal girls Neuropsychiatric disorder Seen in 5 - 15 % cases http://cardiologysearch.blogspot.in/
  • 44. CHOREA ST. VITUS DANCE 25 - 30 % develop RHD particularly MS (Bland et al – 20 years follow up) Multiple purposeless movements of legs and hands (also involves face) on exertion & absent during sleep http://cardiologysearch.blogspot.in/
  • 45. DD FOR CHOREA HABITUAL SPASMS WILSONS DISEASE POST ENCEPHALITIS HYSTERESIS http://cardiologysearch.blogspot.in/
  • 46. SUBCUTANEOUS NODULE FIRM PAINLESS 0.5 – 3 cm IN SIZE IN CROPS ( OVER EXTENSORS) DISAPPEAR IN 12 WEEKSALWAYS ASSOCIATED WITH CARDITIS http://cardiologysearch.blogspot.in/
  • 47. SUBCUTANEOUS NODULEhttp://cardiologysearch.blogspot.in/
  • 48. SUBCUTANEOUS NODULEhttp://cardiologysearch.blogspot.in/
  • 49. ERYTHEMA MARGINATUM Rare (< 1 %) Bikini distribution Evanescent vanishing Non pruritic
  • 50. OTHER MANIFESTATIONS EPISTAXIS ABDOMINAL PAIN- Occurs in 5% cases- Clinical importanceOften appear hours or days before major manifestationsAcute abdomen [ appendicitis ] to be excluded http://cardiologysearch.blogspot.in/
  • 51. FEVER Relatively common But nonspecific Low grade; subside without treatment in 1-2wk Associated with constitutional symptoms Lab indices are high even after fever subsides Remission does not exclude rheumatic activity http://cardiologysearch.blogspot.in/
  • 52. ECG CHANGES Seen in 2/5th patients [ Disciascio(1980)] PR interval ; QT interval ; AV blocks Does not correlate with organic murmurs, prognosis or residual heart disease Nonspecific & occur in many other infection http://cardiologysearch.blogspot.in/
  • 53. LAB INVESTIGATIONSMonitoring the Detecting the antecedentinflammatory activity infection with streptococcus There is no single diagnostic test http://cardiologysearch.blogspot.in/
  • 54. EVIDENCE OF STREPTOCOCAL INFECTIONTH ROAT SWAB CULTURE : Only in Minority of casesASO TITRE :  elevated from 7 - 10 days  rise and fall rapidly  >240 todd units (adults)  >330 todd units (children)  Antibiotics/steroids/liver disease affect the titre http://cardiologysearch.blogspot.in/
  • 55. EVIDENCE OF STREPTOCOCAL INFECTION ANTI-DNAase B TEST : # > 120 todd units (adults) # > 240 todd units (children) # used when ASO titre is not conclusive # remains elevated for long time STREPTOZYME TEST : Detects antibodies against streptococcal antigen http://cardiologysearch.blogspot.in/
  • 56. RHEUMATIC ACTIVITY DETECTION Activity considered ended only when both ESR & CRP become normal and remain so for 2 weeks after stopping drugs Fever & tachycardia subside long before lab reactants decline Joint symptoms & active carditis do not occur after ESR & CRP decline http://cardiologysearch.blogspot.in/
  • 57. RHEUMATIC ACTIVITY DETECTION CRP more specific than ESR Usually lasts for 3 months Longer in patients with valvular involvement In 5% cases rheumatic activity persist longer than 6 months termed CHRONIC RHEUMATIC FEVER http://cardiologysearch.blogspot.in/
  • 58. ECHOCARDIOGRAM Abernathy et al: echo allowed earlier diagnosis of carditis Veasy et al : echo increased the sensitivity of detecting carditis from 72% to 91% http://cardiologysearch.blogspot.in/
  • 59. ECHOCARDIOGRAM Differentiates between innocent murmur and Rheumatic MR Detects MVP due to Rheumatic fever (Wu et al – JACC 1994) - AML - Elongated chordae - No myxomatous thickening http://cardiologysearch.blogspot.in/
  • 60. ECHOCARDIOGRAM Cost effectiveness and the additional workload have to be validated Vasan et al (Circ . 1994 ): showed no additional detection of carditis by echo than by clinical detection http://cardiologysearch.blogspot.in/
  • 61. OTHER INVESTIGTIONSEndomyocardial biopsy – to establish the myocarditisnot likely to provide additional informationsRadionuclide imaging-- Gallium-67 imaging has better diagnostic characteristics than antimyosin scintigraphy - the results confirm that rheumatic carditis is infiltrative rather than degenerative in nature - not suitable for routine investigation http://cardiologysearch.blogspot.in/
  • 62. DUCKETT JONES CRITERIA ORIGINAL (JAMA 1944)MAJOR MINORCarditis erythema mariginatumChorea fever / epistaxis /Arthralgia abdominal painS/C Nodule WBC / ESR / CRPPreexisting RF http://cardiologysearch.blogspot.in/
  • 63. DUCKETT JONES MODIFIED CRITERIA :1956 - AHA Arthritis : Included as – major criteria Erythema marginatum: Included as – major criteria REVISED : 1965 /84 - AHA Recent streptococcal infection is included as essential criteria WHO : 1988 UPDATED : 1992 - AHA WHO CRITERIA : 2003 http://cardiologysearch.blogspot.in/
  • 64. DUCKETT JONES CRITERIA WHO CRITERIA FOR RF AND RHD- 2003 MAJOR MINOR Carditis Clinical Polyarthritis - Fever Chorea - Arthralgia S/C Nodules Laboratory Ery. Marginatum - Leucocytosis - Elevated : ESR /CRP ECG - IncreasedPR interval http://cardiologysearch.blogspot.in/
  • 65. DUCKETT JONES CRITERIASupporting evidence of antecedentstreptococcal infection Within the last 45days - positive Throat culture - Rapid streptococcal antigen test - Elevated or Rising ASO Titer - Recent scarlet fever http://cardiologysearch.blogspot.in/
  • 66. Diagnostic categories: WHO 2003 PRIMARY RF : 2 major or 1 major and 2 minor + evidence of preceding Gr-A streptococcal infection RECURRENT ATTACK OF RF WITHOUT ESTABLISHED RHD 2 major or 1 major and 2 minor + evidence of preceding Gr-A streptococcal infection RECURRENT ATTACK OF RF WITH ESTABLISHED RHD 2 minor + evidence of preceding Gr-A streptococcal infection http://cardiologysearch.blogspot.in/
  • 67. Diagnostic categories: WHO 2003 Rheumatic chorea Insidious onset rheumatic carditis Other major manifestations or evidence of Group-A streptococcal infection not required http://cardiologysearch.blogspot.in/
  • 68. Diagnostic categories: WHO 2003 Chronic valve lesions of RHD Patients presenting first time with pure MS or mixed mitral valve disease and /or aortic valve disease Do not require any other criteria for diagnosis as having RHD http://cardiologysearch.blogspot.in/
  • 69. DUCKETT JONES CRITERIA  Specificity – 97 %  Sensitivity – 77 %http://cardiologysearch.blogspot.in/
  • 70. BEYOND JONES CRITERIA Not a substitute for clinical judgment Not meant to predict course or severity Useful for initial diagnosis only Exceptions : - Chorea - Isolated indolent carditis - Recurrence with RHD http://cardiologysearch.blogspot.in/
  • 71. APPLYING JONES CRITERIA 2 major criteria is stronger than One major and 2 minor Arthalgia cannot be used as minor criteria when arthritis is present Prolonged PR cannot be used as a minor criteria when clinical carditis is present http://cardiologysearch.blogspot.in/
  • 72. APPLYING JONES CRITERIA Absence of evidence of an antecedent Group-A Beta-hemolyticus Streptococci is a warning that RF is unlikely Possibility of early suppression of full clinical manifestations by drugs should be sought during history taking http://cardiologysearch.blogspot.in/
  • 73. RECURRENCE Cardiac status deteriorates with each new attack Younger the patient - higher recurrence rate Recurrence decreases with passage of time – . - 50% within first year - only 10% after 5 years Recurrence more in those with valvular lesion Increase antibody response associated with high recurrence rate http://cardiologysearch.blogspot.in/
  • 74. RECURRENCE Clinical manifestations in recurrence tend to mimic those in preceding attack Recurrence distinguished from rebound or exacerbation if interval of 3 months freedom of rheumatic activity Valve stenosis at diagnosis indicates recurrence http://cardiologysearch.blogspot.in/
  • 75. RHEUMATIC FEVER Licks the Joint and Bites the Heart http://cardiologysearch.blogspot.in/
  • 76. http://cardiologysearch.blogspot.in / http://cardiologysearch.blogspot.in/
  • 77.  THANK YOU http://cardiologysearch.blogspot.in/
  • 78.  Kindly send your suggestions to improve this site Visit us regularly for updates Send your articles/ ppt/pdf to publish in this site . http://cardiologysearch.blogspot.in/ http://cardiologysearch.blogspot.in/