ACUTE RHEUMATIC FEVER

DEFINITION

Rheumatic fever is an inflammatory process which

can involve the joints, heart, skin and brain
It is caused by antibody cross reactivity and occurs
2-3 weeks after a Group A Streptococcal infection.
 EPIDEMIOLOGY

470,000 new cases of Acute Rheumatic Fever/year

233,000 deaths due to Rheumatic Fever/year
Majority of deaths occur in developing countries
Incidence in the US: 2-14 cases/100,000
Historically, there is a temporal relationship between
epidemics of streptococcal pharyngitis and scarlet
fever with the epidemics of acute rheumatic fever
No clear gender predilection overall, but mitral
stenosis and syndenhams chorea occur more in
females than males.
 BACKGROUND

Primarily affects children between ages 5-12 (the

most 8)
Generally occurs 2-3 weeks after Group A
Streptococcal infection (strep throat or scarlet fever)
In the US, Rheumatic fever has become fairly rare due
to use of antibiotics to treat streptococcal infections
Globally, 3% of those with an untreated streptococcal
infection develop rheumatic fever
40% of those with Acute Rheumatic Fever develop
mitral stenosis as adults
 BACKGROUND

Cutaneous streptococcal infections have not been

shown to initiate Acute Rheumatic Fever.
Strains of certain M serotypes/genotypes of
streptococci have higher associations than other
genotypes
 PATHOPHYSIOLOGY

Exact mechanism of how Group A streptococcal

infection causes Acute Rheumatic Fever is unknown
however it is believed to be caused by a cross
reactivity of antibodies
Suggested Theories
Toxic effects of streptococcal products (streptolysin S or O)
which then cause direct tissue injury
Serum Sickness-like reaction mediated by antigen-antibody
complexes
Autoimmune phenomenon
 PATHOPHYSIOLOGY

More support for an autoimmune phenomenon

(Type II hypersensitivity reaction)
During strep infection, antigen presenting cells
present bacterial antigen to helper T cells. These
helper T cells then activate B cells to induce
production of antibodies against the Streptococcal
cell wall. These antibodies can also interact with
other cells in the body (for example, myocardium or
joints, etc) producing the symptoms responsible with
acute rheumatic fever
 PATHOGENESIS

Most patient have elevated antibody titers to at least

one streptococcal antibody
Streptolysin O
Hyaluronidase
Streptokinase
 PATHOPHYSIOLOGY (CARDIAC)

Aschoff nodule with owl-eyed shape in the cross section and catapillar-shaped
in the longitudinal section
PATHOPHYSIOLOGY (CARDIAC)

Thickened fused chordae of the mitral valve

 CLINICAL MANIFESTATIONS

Latent period: time between preceding streptococcal

pharyngitis and Acute Rheumatic fever is about 19
days (range 1-5 weeks)
If initial complaint is polyarthritis, disease generally
has more abrupt onset compared to if initial
presentation is with myocarditis.
Arthritis occurs in 75% of initial attacks, carditis in
40-50% and chorea in 15% with subcutaneous
nodules and erythema marginatum in <10%
CLINICAL MANIFESTATIONS (CARDITIS)

Usually manifests within the first 3 weeks of Acute

Rheumatic Fever
Signs: new heart murmur, cardiomegaly, CHF,
perciardial friction rub, effusions
Chronic inflammatory changes may lead to development
of rheumatic heart disease.
Characteristic murmur or Rheumatic heart disease:
Apical holosystolic murmur and basal early diastolic murmur
Low-pitched mid diastolic flow murmur at the apex (Carey Coombs
murmur
Aortic regurgitation
Can also get AV conduction delays
CARDITIS
Cardiomegaly
Cardiomegaly
primarily prolonged PR interval
AV conduction delays
CLINICAL MANIFESTATIONS (JOINTS)

Arthralgias and arthritis (may be migratory)

Warm, swollen, tender joints
Usually involves the knees, ankles, elbows
and wrists
Lasts 2-3 weeks
Arthralgias and arthritis
 CLINICAL MANIFESTATIONS

Subcutaneous Nodules: usually associated with

severe carditis and occur several weeks after onset.
Firm, painless nodules (up to 2cm) found over bony surfaces
and tendons
Occur near elbows, knees, wrists, achilles tendon, vertebral
joints
Usually persist for 1-2 weeks
20 %
SUBCUTANEOUS NODULES
SUBCUTANEOUS NODULES
 CLINICAL MANIFESTATIONS

Erythema Marginatum: nonpruritic,

painless erythematous rash on trunk
and/or proximal extremities
Macular lesions with raised margins and
central clearing
May last from weeks to months
ERYTHEMA MARGINATUM
 CLINICAL MANIFESTATIONS

Sydenhams Chorea: neurologic disorder with

muscular weakness, emotional lability and
involuntary, uncoordinated, purposeless
movements
Disappear during sleep
Mainly occur in hands, feet and face

Sensation intact

Lasts 2-4 months

5-36%

Girls
Sydenhams Chorea
 DIFFERENTIAL DIAGNOSIS

Poststreptococcal reactive arthritis: is non-migratory

Rheumatoid Arthritis
SLE
Infective endocarditis
Sickle Cell disease
Drug reactions
TB
Lyme Disease
Serum Sickness
 DIAGNOSIS

JONES CRITERIA
Developed by Dr. T Duckett Jones in 1944
Need 2 major criteria or 1 major and 2 minor criteria in the
presence of a prior strep infection to make the diagnosis
Evidence of prior strep infection with positive throat culture or
antigen test, elevated streptococcal antibody titer, or history of
rheumatic fever/heart disease
 MAJOR CRITERIA

Migratory Polyarthritis: migrating arthritis with

inflammation involving the large joints (knees, ankles,
elbows, wrists) and typically affects the leg joints first
Carditis: can manifest with new murmur, pericarditis,
congestive heart failure
Subcutaneous Nodules: a form of aschoff bodies. Are
painless nodules on the back of the wrists, elbows, knees
Erythema Marginatum: rash beginning on the arms or
trunk and spreads outward. Lesion with ring with central
clearing. Worsens with heat. Does not involve the face
Sydenhams Chorea (St. Vitus Dance): purposeless
movements of the face and arms. Late finding
 MINOR CRITERIA

Fever
Arthralgia: joint pain without inflammation
Elevated CRP, ESR or leukocytosis
EKG changes: primarily prolonged PR interval
Evidence of Group A Streptococcal infection via
elevated antistreptolysin O titer or DNAase
ASTO > 120-140 IU, dan anti deokribonukleas > 60-
600
Prior history of rheumatic fever or heart disease
 Diagnostik kriteria
Demam reumatik Dua mayor atau satu
Dasar serangan pertama mayor dan dua minor
Diagnosis ditambah dengan bukti
infeksi streptococcus Beta
Kriteria WHO thn
2002-2003 hemolitikus group A
berdasarkan revisi Demam reumati serangan Sama dengan diatas
kriteria Jones
ulangan tanpa PJR
Demam reumatik Dua minor ditambah
serangan ulangan dengan dengan bukti infeksi beta
PJR streptococus hemolitikus
Korea reumatik Tdk perlu kriteria mayor
lain atau bukti infeksi
 TREATMENT

Anti-inflammatory Agents
Antibiotics
Prophylaxis
 ANTI-INFLAMMATORY AGENTS

Aspirin 90 -100 mg/kgBB/day --2 weeks and

tappering 25 mg/kgBB/day---4-6 weeks
Continue anti-inflammatory therapy until ESR or
CRP are normal
May need steroids if there is cardiac involvement to
help prevent sequelae such as mitral stenosis
Corticosteroids, if indicated, are given at prednisone
2mg/kg/day for 2 weeks and then tapered
 ANTIBIOTICS

Penicillin for at least 10 days

Benzatin penicilin G
BB < 30 kg : 600.00 IU im
BB > 30 kg : 1.200.000 IU im
Can use erythromycin for PCN allergic patients
(given at 40mg/kg/day given in 2-4 doses/day)
Penicilin V 4 X 250 mg PO
 Anti Inflamasi
Manifestasi Klinis terapi
Atralgia Paracetamol
Arhritis Salisilat
Karditis Prednison 2 mg/kgBB/day
dibagi 4-6 dosis PO 2
minggu-tappering off 2
mg
Salisilat 75 mg/kgBB/day
selama 2 minggu, lalu
tappering 60 mg/kgBB/hari
 Panduan obat anti inflmasi

Arthritis Karditis Karditis Karditis

ringan sedang berat
Prednison 0 0 2-4 mg 2-6 mg
aspirin 1-2 mg 2-4 mg 6-8 mg 2-4 bl
 Terapi korea Sydenham

Pengurangan aktivitas dan gangguan emosi

Benzatin penisilin G
Anti inflamasi tidak diberikan
Kasus berat diberikan haloperidol, asam valproat,
diazepam
 Panduan Tirah Baring dan aktivitas

aktivitas Arthritis Karditis Karditis Karditis

minimal sedang berat

Tirah baring 1-2 mg 2-3 mg 4-6 mg 2-4 bl

Aktivitas 1-2 mg 2-3 mg 4-6 mg 2-3 bl

dalam rumah

Aktivitas di 2 mg 2-4 mg 1-3 bl 2-3 bl

luar rmh

Aktivitas Ssdh 6-10 mg Sesudah 6-10 Ssdh 3-6 bl bervariasi

penuh mg
 PROPHYLAXIS

Prophylaxis needed to prevent recurrence of Acute

Rheumatic Fever
Start prophylaxis after acute episode resolves
Can use:
Penicillin V 250mg BID or,
Sulfadiazine 1000mg daily, or
Penicillin G 1.2 million units IM q4weeks
For PCN allergic patients: erythromycin 250mg PO BID
Recurrence of disease generally occurs in the first
couple years
 PROPHYLAXIS

WHO GUIDELINES
At least 5 years of prophylaxis or if child until age 18 if not
cardiac involvement
10 years prophylaxis or if child until age 25 if has mild mitral
regurgitation
Lifelong prophylaxis if has severe valve disease
 PROPHYLAXIS

Primer
Terapi antibiotik secara adekuat thd infeksi sal napas atas oleh
strptococcus group A
Sekunder
Pemberian antibiotik spesifik secara kontinyu pada penderita
DRA dan PJR
 Lama pemberian profilaksis sekunder

Kategori penderita Durasi

Tanpa karditis Selama 5 thn sesudah serangan terakhir
atau usia 18 thn
Dengan karditis (MI atau karditis Seama 10 th setelah serangan atau
perbaikan) sampai usia 25 thn
PJR Seumur hidup
Pasca bedah katup Seumur hidup
Complications
Complications
Complications