Se ha denunciado esta presentación.

Acute rheumatic fever - Dr. Akif Baig

0

Compartir

Próximo SlideShare
Ms mr rhd
Ms mr rhd
Cargando en…3
×
1 de 81
1 de 81

Acute rheumatic fever - Dr. Akif Baig

0

Compartir

Descargar para leer sin conexión

An acute illness caused by an autoimmune response to infection with group A Streptococcus, leading to a range of possible symptoms and signs affecting any or all of heart, joints, brain, skin and subcutaneous tissues

An acute illness caused by an autoimmune response to infection with group A Streptococcus, leading to a range of possible symptoms and signs affecting any or all of heart, joints, brain, skin and subcutaneous tissues

Más Contenido Relacionado

Libros relacionados

Gratis con una prueba de 30 días de Scribd

Ver todo

Audiolibros relacionados

Gratis con una prueba de 30 días de Scribd

Ver todo

Acute rheumatic fever - Dr. Akif Baig

  1. 1. Acute Rheumatic Fever - Dr. Akif A.B
  2. 2. DEFINITION  Acute rheumatic fever  An acute illness caused by an autoimmune response to infection with group A Streptococcus, leading to a range of possible symptoms and signs affecting any or all of heart, joints, brain, skin and subcutaneous tissues  Rheumatic carditis  Active inflammation of the heart tissues, most importantly the mitral and/or the aortic valves, caused by acute rheumatic fever. Rheumatic carditis can lead to chronic damage that remains after the acute inflammatory episode has resolved  Rheumatic heart disease  The persistent damage to heart valves resulting in mitral and/or aortic regurgitation, or in long-standing cases stenosis, that remains as a result of acute rheumatic fever with rheumatic carditis. Complications of rheumatic heart disease include heart failure, embolic stroke, endocarditis and atrial fibrillation.
  3. 3. EPIDEMIOLOGY  More than 2.4 million children have rheumatic heart disease worldwide; 94% of these are in developing countries  Worldwide there are over 330,000 new cases of acute rheumatic fever each year  Overall, it is estimated that there are over 25 million people affected by rheumatic heart disease leading to 345,000 deaths per year
  4. 4. Epidemiology of Rheumatic Fever in India
  5. 5.  The burden of RF/RHD has been estimated and reported since 1960s from hospital-based, population-based [Table 2], and school-based survey studies, using different case definitions and screening methods.  There are no survey studies estimating the burden of RF/RHD based on national- and state representative sample using uniform screening method at the different timeline to evaluate the trends of the burden of RF/RHD in India
  6. 6. HOSPITAL BASED STUDIES
  7. 7. HOSPITAL BASED STUDIES  Hospital admission data show a decline in admission rates of RF/RHD overtime period. RF/RHD accounted for 30%e50% of total admissions until the early 1980s, and it declined to 5% -26% in the late 1990s  Whether the declining trends in admission rate truly reflects the decreasing incidence is much to be debated  Inadequacy of hospital statistics, varied hospital admission policies, and a large number of corporate hospitals coming up can cause significant bias in hospital- based data  The emergence of an epidemic of coronary artery disease (CAD) and lack of interest among cardiologists in RHD has further compounded the problem  The only useful forgone conclusion can be derived from hospital statistics if the data are derived from the same hospital over a different time period  A study from territory care center in Orissa showed no change in admission rate over a decade and the admission rate of 50% in 2013
  8. 8. Population-based survey studies  Data from population-based surveys are likely to give us reliable estimates of the prevalence of RF/RHD  There are no data available about the prevalence of RF/RHD based on active surveillance studies in a representative sample of the country or the state  The available data are based on estimation performed in cities or rural areas of certain regions of the states in different points of time using either clinical screening method alone or confirmed by echocardiography
  9. 9. SCHOOL BASED SURVEYS
  10. 10. Survey studies with clinical screening method (period 1960s to 1990s)  The estimation of prevalence of RF/RHD among school children performed in the period from 1970s until 1990s was based on clinical screening method alone  Thus, reported figures of prevalence have the limitation of sensitivity and specificity of the cases reported
  11. 11. Survey studies with clinical screening confirmed by echocardiography (period 1990s to 2000)  The epidemiological studies with clinical screening followed by confirmation of suspected cases with echocardiography using Doppler- based World Health Organization criteria in urban and rural school children in different regions of the country from early 90s to early 2000s reported prevalence ranging from 1.3/1000 to 6.4/1000  The reported variation in the prevalence of RF/RHD may be an indication of a varied burden of RF/RHD across different regions, urban, rural areas, and/or temporal trends apart from methodological-related factors
  12. 12. ESTIMATED BURDEN OF DISEASE  From available data from RHD studies, the estimated average prevalence is 0.5/1000 children in age group of 5-15 years  There are expected to be more than 3.6 million patients of RHD estimated from 2011 census  Almost 44,000 patients are added every year, and expected mortality is 1.5%-3.3% per year  These figures still may be the underestimation of disease as no data are available from large populous, underdeveloped states such Bihar, Jharkhand, and so on
  13. 13.  Low Risk  Children aged 5–14 years living in a community with an incidence of RF of <2/100,000/year or  Any age where the prevalence of chronic rheumatic carditis is one or more/1000 per year are considered low risk  High Risk  Children living in areas with an incidence of two or more/100,000/year in children aged 5–14 years  Prevalence of chronic rheumatic carditis more than one/1000/year at any age are considered at a moderate to high risk of developing the disease
  14. 14. RISK FACTORS
  15. 15. AGE  The incidence of initial cases of ARF is highest in children aged 5–14 years, although first episodes do occur in younger children, with reported cases of ARF in those as young as 2–3 years old  Initial episodes can also occur in older adolescents and adults, although cases in people >30 years of age are rare  By contrast, recurrent episodes often affect slightly older children, adolescents and young adults but are rarely observed beyond the age of 35–40 years
  16. 16. AGE  RHD is a chronic disease caused by accumulated heart valve damage from a single severe or, more commonly, multiple recurrent ARF episodes  This means that, although RHD occurs in children, its prevalence peaks in adulthood, usually between the ages of 25 years and 45 years
  17. 17. SEX  ARF is equally common in males and females  RHD occurs more commonly in females, with a relative risk of 1.6 to 2.0 compared with males  In addition, these sex differences might be stronger in adolescents and adults than in children
  18. 18.  The reasons for this association are not clear, but intrinsic factors such as greater autoimmune susceptibility, as observed in systemic lupus erythematosus  Extrinsic factors such as greater exposure to GAS infection in women than in men as a result of closer involvement in child-rearing might explain this difference  In addition, women and girls might experience reduced access to primary and secondary ARF prophylaxis compared with men and boys, and this could also contribute to differences in RHD rates between females and males
  19. 19. Environmental factors  Poverty and economic disadvantage  Household overcrowding is perhaps the best described risk factor and reduced overcrowding has been cited as one of the most important factors underlying the decline in ARF incidence in wealthy countries during the twentieth century
  20. 20. ORGANISM FACTORS  ARF is exclusively caused by infection of the upper respiratory tract with group A streptococci  Although classically, certain M-serotypes (particularly types 1, 3, 5, 6, 14, 18, 19, 24, 27, and 29) were associated with ARF, in high- incidence regions, it is now thought that any strain of group A streptococcus has the potential to cause ARF  The potential role of skin infection and of groups C and G streptococci is currently being investigated
  21. 21. HOST FACTORS  Approximately 3–6% of any population may be susceptible to ARF  44% concordance in monozygotic twins compared to 12% in dizygotic twins  Some human leukocyte antigen (HLA) class II alleles, particularly HLA-DR7 and HLA-DR4, appear to be associated with susceptibility  Whereas other class II alleles have been associated with protection (HLA-DR5, HLA-DR6, HLA-DR51, HLA-DR52, and HLA-DQ)
  22. 22. IMMUNE RESPONSE  Most widely accepted theory of rheumatic fever pathogenesis is based on the concept of molecular mimicry, whereby an immune response targeted at streptococcal antigens (mainly thought to be on the M protein and the N- acetylglucosamine of group A streptococcal carbohydrate) also recognizes human tissues  An alternative hypothesis proposes that the initial damage is due to streptococcal invasion of epithelial surfaces, with binding of M protein to type IV collagen allowing it to become immunogenic, but not through the mechanism of molecular mimicry.
  23. 23. Pathogenesis  After GAS infection of the pharynx, neutrophils, macrophages and dendritic cells phagocytose bacteria and present antigen to T cells  Both B and T cells respond to the GAS infection, initially by antibody production (IgM and IgG) and subsequently through T cell activation (mainly CD4+ cells)  In susceptible individuals, the host response against GAS will trigger autoimmune reactions against host tissues mediated by both Streptococcus spp.-specific antibodies and T cells through a process called molecular mimicry
  24. 24. PATHOLOGY
  25. 25. CLINICAL FEATURES
  26. 26.  There is a latent period of ~3 weeks (1–5 weeks) between the precipitating group A streptococcal infection and the appearance of the clinical features of ARF  The exceptions are chorea and indolent carditis, which may follow prolonged latent periods lasting up to 6 months  Although many patients report a prior sore throat, the preceding group A streptococcal infection is commonly subclinical; in these cases, it can only be confirmed using streptococcal antibody testing
  27. 27.  The most common clinical features are polyarthritis (present in 60–75% of cases) and carditis (50–60%)  The prevalence of chorea in ARF varies substantially between populations, ranging from <2 to 30%  Erythema marginatum and subcutaneous nodules are now rare, being found in <5% of cases
  28. 28. HEART INVOLVEMENT  Up to 60% of patients with ARF progress to RHD.  The endocardium, pericardium, or myocardium may be affected  Valvular damage is the hallmark of rheumatic carditis  The mitral valve is almost always affected, sometimes together with the aortic valve; isolated aortic valve involvement is rare.  Damage to the pulmonary or tricuspid valves is usually secondary to increased pulmonary pressures resulting from left-sided valvular disease.
  29. 29. Valve involvement Prevalance Isolated MS 25% Combined MS and MR 40% MS + Aortic Valve 35% MS + Tricuspid valve involvement 6%
  30. 30.  Early valvular damage leads to regurgitation.  Over ensuing years, usually as a result of recurrent episodes,leaflet thickening, scarring, calcification, and valvular stenosis may develop  Therefore, the characteristic manifestation of carditis in previously unaffected individuals is mitral regurgitation, sometimes accompanied by aortic regurgitation  Myocardial inflammation may affect electrical conduction pathways, leading to P-R interval prolongation (first-degree atrioventricular block or rarely higher level block)
  31. 31. JOINT INVOLVEMENT  Joint involvement is seen in 70% of patients  The joint pain is typically described as “migratory,” which refers to the sequential involvement of joints, with inflammation resolving in one joint and then beginning in another joint  In some cases the joint involvement may be additive rather than migratory, with simultaneous involvement of several joints
  32. 32.  The affected joint may be inflamed for only a few days to 1 week before the inflammation subsides  The polyarthritis is severe for approximately 1 week in two thirds of patients and may last another 1 to 2weeks in the remainder before it resolves completely  If joint swelling persists after 4 weeks, it becomes necessary to consider other conditions, such as juvenile idiopathic arthritis or systemic lupus erythematosus (SLE)  At the onset of the illness the joint involvement is asymmetric and usually affects the lower limbs initially before spreading to the upper limbs  Monoarthritis has been reported in 17% to 25% of patients  The large joints such as the knees, ankles, elbows, and wrists are most frequently involved
  33. 33. Jaccoud arthritis or arthropathy  chronic post–rheumatic fever arthropathy is a rare manifestation of rheumatic fever characterized by deformities of the fingers and toes  There is ulnar deviation of the fingers, especially the fourth and fifth fingers, flexion of the metacarpophalangeal joints, and hyperextension of the proximal interphalangeal joints (i.e., swan neck deformity)  The hand is usually painless, and there are no signs of inflammation  The deformities are usually correctible but may become fixed in the later stages  There are no true erosions on radiography, and the rheumatoid factor is usually negative
  34. 34. CHOREA  Sydenham’s chorea commonly occurs in the absence of other manifestations  Prolonged latent period after group A streptococcal infection, and is found mainly in females  The choreiform movements affect particularly the head (causing characteristic darting movements of the tongue) and the upper limbs  They may be generalized or restricted to one side of the body (hemi-chorea)  Lasts from 6weeks to 6 months
  35. 35. ERYTHEMA MARGINATUM  It begins as pink macules that clear centrally, leaving a serpiginous, spreading edge  The rash is evanescent, appearing and disappearing before the examiner’s eyes  It occurs usually on the trunk, sometimes on the limbs, but almost never on the face
  36. 36. SUBCUTANEOUS NODULES  Subcutaneous nodules occur as painless, small (0.5–2 cm), mobile lumps beneath the skin overlying bony prominences, particularly of the hands, feet, elbows, occiput, and occasionally the vertebrae  They are a delayed manifestation, appearing 2–3 weeks after the onset of disease, last for just a few days up to 3 weeks, and are commonly associated with carditis
  37. 37.  Fever occurs in most cases of ARF, although rarely in cases of pure chorea  Although high-grade fever (=39°C) is the rule, lower grade temperature elevations are not uncommon  Elevated acute-phase reactants are also present in most cases
  38. 38. DIAGNOSIS
  39. 39. EVOLUTION OF JONES CRITERIA
  40. 40. Erythema marginatum was elevated to a major status, and evidence of preceding beta-hemolytic Streptococcal infection was accorded minor status Minor criteria (abdominal and precordial pain, epistaxis, pulmonary findings and anemia) were Dropped The most prominent difference, however, was the downgrading of arthralgia and previous rheumatic fever or rheumatic heart disease to a minor status
  41. 41. REVISED JONES CRITERIA- 1965  Distinguishing feature of this revision as the necessity of demon strating evidence of recent streptocoecal infection.  This followed the recognition of the streptococcal etiology of rheumatic fever, and of the fact that streptococcal antibodies could be demonstrated in the great majority of patients with rheumatic fever
  42. 42. EVIDENCE OF A PRECEDING GROUP A STREPTOCOCCAL INFECTION  With the exception of chorea and low-grade carditis, both of which may become manifest many months later, evidence of a preceding group A streptococcal infection is essential in making the diagnosis of ARF  Because most cases do not have a positive throat swab culture or rapid antigen test, serologic evidence is usually needed  The most common serologic tests are the anti- streptolysin O (ASO) and anti-DNase B (ADB)
  43. 43.  The antistreptolysin O test is usually obtained first, and if it is not elevated, an antideoxyribonuclease B test may be performed  Antistreptolysin O titers begin to rise approximately 1 week and peak 3 to 6 weeks after the infection  Antideoxyribonuclease B titers begin to rise 1 to 2 weeks and peak 6 to 8 weeks after the infection
  44. 44. Raised antistreptolysin O level (ASO)  Rheumatic fever  Glomerulonephritis  Endocarditis.  Scarlet fever  Titres >200IU (160 Todd Units)
  45. 45. TREATMENT
  46. 46.  The aim of treatment of a proven attack of rheumatic fever is:  To suppress the inflammatory response and thus minimize the effects of inflammation on the heart and joints  To eradicate the GAS from the pharynx  To provide symptomatic relief  To commence secondary prophylaxis
  47. 47. Unconfirmed diagnosis, presenting with monoarthritis  If acute rheumatic fever is suspected, admission to the hospital for observation and further investigation is indicated  Salicylate therapy is usually withheld pending confirmation of the diagnosis, and simple analgesics such as paracetamol or codeine are recommended in the interim  Salicylates are withheld to facilitate diagnosis; they reduce arthritic pain but do not affect the long-term outcome of the disease
  48. 48. Confirmed acute rheumatic fever  The outcome of rheumatic valvular lesions has not been shown to be affected by the administration of penicillin during an episode of acute rheumatic fever  However, penicillin is recommended by the authors to ensure eradication of streptococci in the throat whether there has been a positive throat culture or not  The choice is either oral phenoxymethylpenicillin (penicillin V) for 10 days or a single injection of benzathine benzylpenicillin  As penicillin is the first-line choice for secondary prophylaxis it is recommended that a patient with reported penicillin allergy be investigated carefully
  49. 49. ANTIBIOTICS  Even though throat swabs taken during the acute attack of rheumatic fever are rarely positive for GAS, it is advisable for patients to receive  Thereafter, secondary prophylaxis should be commenced All patients with ARF should receive antibiotics sufficient to treat the precipitating group A streptococcal infection  Penicillin is the drug of choice Antibiotics Dose Duration Benzathine penicillin G 1.2 million units (600,000 units for children ≤27 kg) Single dose I.M Amoxicillin 50 mg/kg (maximum, 1 g) 10days Phenoxymethyl penicillin (Penicillin V) 500 mg [250 mg for children ≤27 kg] PO twice daily 10days
  50. 50. CARDITIS  Most patients with mild or moderate carditis without cardiac failure do not require any therapy  A subset of patients with carditis who develop cardiac failure do require treatment:  Bed rest with ambulation as tolerated  Medical management of heart failure; first-line therapy is diuretics, and ACE inhibitors may be added in severe heart failure or where aortic regurgitation is present  Despite the absence of high-quality evidence to support the use of glucocorticoid therapy for patients with carditis and severe heart failure, there is consensus among clinicians treating rheumatic fever that the use of glucocorticoids can speed recovery  Two meta-analyses have failed to show any benefit of glucocorticoids over placebo, although contributing studies were old and generally of low quality  There is no evidence that salicylates or intravenous immunoglobulin (IVIG) improve the outcome from carditis in rheumatic fever and we do
  51. 51. BED REST  The longstanding recommendation of bed rest would appear to be appropriate mainly to lessen joint pain  The duration of bed rest should be individually determined, but ambulation can usually be started once the fever has subsided and acute- phase reactants are returning to normal  Strenuous exertion should be avoided, especially for those with carditis
  52. 52. SALICYLATES AND NSAIDs  These may be used for the treatment of arthritis, arthralgia, and fever, once the diagnosis is confirmed  They are of no proven value in the treatment of carditis or chorea  Aspirin is the drug of choice, delivered at a dose of 50–60 mg/kg per day, up to a maximum of 80–100 mg/kg per day (4–8 g/d in adults) in 4– 5 divided doses  Fever, joint manifestations, and elevated acute phase reactants sometimes recur up to 3 weeks after the medication is discontinued  This does not indicate a recurrence and can be managed by recommencing salicylates for a brief period  Naproxen at a dose of 10–20 mg/kg per day is a suitable alternative to aspirin and has the advantage of twice-daily dosing
  53. 53. CHOREA MANAGEMENT  Medications to control the abnormal movements do not alter the duration or outcome of chorea  Milder cases can usually be managed by providing a calm environment  In patients with severe chorea, carbamazepine or sodium valproate is preferred to haloperidol  A response may not be seen for 1–2 weeks, and medication should be continued for 1–2 weeks after symptoms subside  There is recent evidence that corticosteroids are effective and lead to more rapid symptom reduction in chorea
  54. 54. PROGNOSIS  Untreated, ARF lasts on average 12 weeks  With treatment, patients are usually discharged from hospital within 1–2 weeks  Inflammatory markers should be monitored every 1–2 weeks until they have normalized (usually within 4–6 weeks), and an echocardiogram should be performed after 1 month to determine if there has been progression of carditis
  55. 55. PRIMARY PROPHYLAXIS  The mainstay of primary prevention for ARF remains primary prophylaxis (i.e., the timely and complete treatment of group A streptococcal sore throat with antibiotics)  If commenced within 9 days of sore throat onset, a course of penicillin (as outlined above for treatment of ARF) will prevent almost all cases of ARF
  56. 56.  ARF can recur as a result of subsequent GAS infections and each recurrence can worsen RHD  Thus, the priority in disease management is to prevent ARF recurrences using long-term penicillin treatment, which is known as secondary prophylaxis
  57. 57. SECONDARY PROPHYLAXIS  The best antibiotic for secondary prophylaxis is benzathine penicillin G (1.2 million units, or 600,000 units if ≤27 kg) delivered every 4 weeks  It can be given every 3 weeks, or even every 2 weeks, to persons considered to be at particularly high risk, although in settings where good compliance with an every-4-week dosing schedule can be achieved, more frequent dosing is rarely needed  Oral penicillin V (250 mg) can be given twice daily instead but is less effective than benzathine penicillin G  Penicillin-allergic patients can receive erythromycin (250 mg) twice daily.
  58. 58. AHA Recommendations
  59. 59. WHO Criteria

×