recent udate in

1. ACUTE RHEUMATIC FEVER
Speaker: Dr Ramdhan kumar
Moderator: Dr Dipankar Ghosh
Dastidar

2. ACUTE RHEUMATIC FEVER
 It is multisystem disease.
 ARF results from an autoimmune
response to infection with group A
streptococcus.
 Major effect on health is due to
damage to heart valves.

3.  Occurring most often between 5 and 15 years.
 The inflammatory process causes damage to collagen fibrils and
connective tissue ground substance, resulting in combinations
of
 Arthritis,
 Carditis,
 Erythema marginatum,
 Subcutaneous nodules, and
 Chorea.
 The French physician Ernst-Charles Lasegue famously said, in
1884, that “rheumatic fever licks the joints but bites the heart.”

4. EPIDEMIOLOGY
TheWorldwide, incidence is 19/100,000 (range, 5 to 51/100,000), with
lowest rates (<10/100,000) in North America and Western Europe and
highest rates (>10/100,000) in Eastern Europe, the Middle East, Asia,
Africa, Australia, and New Zealand.2

5. AFRICA, SOUTH ASIA
POSTANTIBIOTIC ERA

7. Its occurrence is similar between women and men,
the inherent biological factors, the risk of illness
during pregnancy, exposure to GAS through child
rearing, and poor accessibility to resources make
women approximately 1.8 times more susceptible
to developing RHD.3

8. PATHOGENESIS
RF is a multifactorial disease that follows GAS (the agent)
pharyngitis in a susceptible individual (the host) who lives under
deprived social conditions (the environment).
The theory of molecular mimicry hold that GAS pharyngitis triggers
an autoimmune response in susceptible individuals by cross-reacting
with similar epitopes in the heart, brain, joints, and skin, and that
repeated episodes of RF lead to RHD. In situations of untreated
epidemic GAS pharyngitis, up to 3% of patients develop the disease.

9. THE HYPOTHESIS OF MOLECULAR MIMICRY:
In the pathogenesis of RF1 state that patients with RHD
have cross-reactive autoantibodies that target the
dominant GAS epitope of the group A carbohydrate, N-
acetyl-beta-d-glucosamine (GlcNAc), and laminin and
laminar basement membrane in heart valve
endothelium. T cells in peripheral blood and heart
valves of RHD patients cross-react with streptococcal M
protein and cardiac myosin.

10. THE TWO-HIT HYPOTHESIS:
For the initiation of disease proposes that antibody
attack of valve endothelium facilitates the
extravasation of T cells through activated epithelium
into valve tissue, leading to the formation of
granulomatous nodules called Aschoff bodies,
characteristic of rheumatic myocarditis .The area of
central necrosis is surrounded by a ring of plump
histiocytes, called Anitschkow cells. These nodules
were discovered by Ludwig Aschoff and Paul Rudolf
Geipel and thus are occasionally called Aschoff-

15. DIAGNOSIS OF ACUTE RHEUMATIC FEVER

16. Revised Jones Criteria
The 2015 revised Jones criteria incorporate subclinical valvulitis
detected by echocardiography is accepted as a major criterion for the
diagnosis of ARF in all patient populations for the first time.
Two sets of criteria introduced based on population risk;
LOW RISK POPULATION: ARF incidence <2/100,000 school
age children per year or RHD prevalence of <1/1000.
MODERATE AND HIGH RISK: ARF incidence_>2/100,000school
age children per year or an all age RHD prevalence of >1 /1000.

18. POLYARTHRITIS

19. POLYARTHRITIS
 Most common manifestation (65-75%)
 More common and more severe in young adults (100%), teenagers (82%), and children (66%).
 Asymmetric, migratory, non suppurative and self limited.
 In some cases involvement may be additive rather than migratory, with several joints affected simultaneously.
 Large joints involved (knee, ankle, elbow, and wrist)
 Shoulder, hip, and small joints of hands and feet may also involved, but almost never alone.
 If vertebral joints are affected, another disorder should be suspected.
 It resolve completely, if the joint swelling persist after 4 weeks, consider other condition, such as JIA or SLE.
 Salicylates and NSAIDS produce a marked and promt relief.
 MONOARTHRITIS:
Occurs in high risk indigenous population (e.g. in India ,Australia, Fiji)- 17 to 25% of patiants.

20. Jaccoud arthritis or arthropathy (or chronic post-RF arthropathy):
 Is a rare manifestation of RF characterized by deformities of the fingers and
toes.
 The condition may occur after repeated attacks of RF and results from
recurrent inflammation of the fibrous articular capsule.
 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 usually correctible but may become fixed in the later stages.
There are no true erosions on radiography, and the rheumatoid factor is
usually negative.

22. Poststreptococcal reactive arthritis (PSRA):
 Is diagnosed in patients who have arthritis that is not typical of
RF but who have evidence of recent streptococcal infection.
 This condition occurs after a shorter latent period than RF, is less
responsive to NSAIDs, may be associated with renal
manifestations, and evidence of carditis is infrequent.
 The distinction between PSRA and RF is unclear, and many
would recommend that a diagnosis of PSRA not be made in
populations where RF is common. Even if the diagnosis is
considered, it is appropriate to offer a period of secondary
prophylaxis with penicillin, as for episodes of acute RF, in such
populations.

23. Carditis
 The incidence of carditis during the initial attack of RF varies from 40% to 91%.
 Carditis is the most serious manifestation of RF because it may lead to chronic RHD.
 It may be asymptomatic and detected during clinical examination of patients with arthritis or
chorea.
 Heart failure results from a combination of carditis and valvular dysfunction and occurs in 5%
to 10% of the initial episodes, more frequently during recurrences of RF.
 Patients may have high fever, chest pain, or both; tachycardia is common, especially during
sleep.
 In about 50% of cases, cardiac damage (i.e., persistent valve dysfunction) occurs much later.
 The symptoms and signs depend on whether there is involvement of the pericardium,
myocardium, or heart valves.
 Valvulitis is the most consistent feature of ARF, and if it is not present, the diagnosis should be

28. Echocardiography is recommended for all patients with suspected or definite ARF, as it is
more sensitive and specific than cardiac auscultation for detection of acute rheumatic
carditis.

31. A score of 5 or more was found to be best
threshold for diagnosis of carditis (ROC AUC
0.87, sensitivity 76%, specificity 79%).

35. Sydenham Chorea
 Sydenham chorea, also referred to as St. Vitus dance, consists of rapid, involuntary, purposeless,
and irregular jerking movements that may begin in the hands but often become generalized,
involving the feet and face and interfering with voluntary activity; they disappear during sleep.
 The CNS is affected in up to 40% of children with RF,23 predominating in females after puberty.
 The latent period between GAS pharyngitis and chorea is longer (6 to 8 weeks) than for arthritis
and carditis;
 its onset is typically insidious and may be preceded by inappropriate laughing or crying.
 It can last for up to 2 years (usually 8 to 15 weeks);
 if it occurs in isolation, all inflammatory markers may be normal and the diagnosis may be
overlooked as an indicator of ARF.
 It does not occur simultaneously with arthritis but may coexist with carditis.
 Usally self-limiting.

37. Cutaneous and Subcutaneous
Features
 Rarely, subcutaneous nodules and erythema marginatum
develop in patients already having carditis, arthritis, or chorea;
 They almost never occur alone.
Subcutaneous nodules
 Occur most frequently on the extensor surfaces of large joints.
 They may be detected over the occiput, elbows, knees, ankles,
and Achilles tendons.
 Ordinarily, the nodules are firm, painless, and freely movable
over the subcutaneous tissue;
 They vary in size from 0.5 to 2 cm and tend to occur in crops that
may be related to the severity of the carditis.
 They are transitory (seldom more than 1 month) and respond to
treatment of joint or heart inflammation.

38. Erythema Marginatum
 Painless rash in fewer than 6% of children.
 The rash usually appears on the trunk and proximal extremities but not on the face.
 It is evanescent, pink, and nonpruritic.
 It extends centrifugally.
 It sometimes lasts less than 1 day and may become more prominent after a shower/hot
bath.

42. Differential Diagnosis
JIA (especially systemic and, less so, polyarticular),
Lyme disease,
Reactive arthritis,
Arthropathy of sickle cell disease,
Leukemia or other cancer,
SLE,
Embolic bacterial endocarditis,
Serum sickness,
Kawasaki disease,
Drug reactions,
Diurnal variation of the fever, evanescent rash, and prolonged symptomatic joint
inflammation usually distinguish systemic JIA from ARF.

43. NATURAL HISTORY
Patients who have had RF have about a 50% likelihood of having a recurrence if
they have another episode of untreated GAS pharyngitis.
In endemic areas for RHD it is usual to see patients with severe RHD and
superimposed ARF, particularly carditis.
Episodes of Sydenham chorea usually last several months and resolve
completely in most patients, but about one-third of patients have recurrences.
Joint inflammation may take one month to subside if not treated but does not
lead to residual damage.
Prognosis following an episode of ARF depends mostly on how severely the
heart is affected, and whether it is a recurrent episode of ARF.
Murmurs disappear in about half of patients whose acute episodes were
manifested by mild carditis without major cardiac enlargement or
decompensation; however, chronic valvular disease can occur, typically over
years or decades, in patients who recovered from the acute episode with no
evidence of valvular disease.
Chronic RHD is the cause of 25% to 45% of all cardiovascular disease and a
major cause of heart failure in developing countries.

55. INJECTABLE VS ORAL PENCILLIN……

59. THANK YOU