Rheumatic Fever

Definition

Inflammatory disease (Strep. Pharyngeal

Group A)
• Heart
• Joints
• CNS
• Skin and Subcutaneous tissue
• Non of signs, symptoms & lab exam
are pathognomonic of RF
• but combination of symptoms helpful
for Dx.
Etiology

• Unknown etiology
• but the relation between Rheumatic
fever and Streptococc is clearly
known.
Pathogenesis

• Upper respiratory infection and Sore throat

1-3 week before attack of RF
• During attack of RF ↑ in ASO titer and
Fibrinolysin
• Strep causes relapse of RF
• Treatment of acute attack of RF with
antibiotics
• Prophylaxis of attack of RF with antibiotics
Cont…
• β-Hemolytic streptococc
– A-B-C-D-F-G
– Group A is more pathogen for human & animals
• More than 50 types
• M-protein
– Sever Virulence & resistant to phagocytosis
• Rheumatogenic M-Protein- serotype

• Skin strep. Infections not causing RF

• There is no clear mechanism that Strep. A cause RF
Continue

• With Fluorescent antibody

– Gamma globulin is found in Sarcolemma of
myocard of patient which is died because of
Rheumatic carditis.

– Gama globulin is found in atrial appendage

of Mitral stenosis pts who goes for Mitral
valve surgery
Incidence
• All ages
• Peak incidence in (5 – 15) year
• Seldom in infants
• Incidence and severity is due to:
– Strept. pharyngitis
Cont..

• Streptococcal pharyngitis group A

– Common (Type 5) // rarely type 12
– Geographical Status
– Humidity
– Bacterial
– Environmental
– Host factors
– Economical
– Age
Cont..

• Risk of RF after strep. In pts with

pervious RF is 5-50% and it belong to
virulence of bacteria.
• Relapse of RF is always in pts with RHD
is more sever, than pts who’s heart not
affected in the with pervious attacks.
Cont..

• After few years the relapse of RF due

to strep. is decline.
• Incidence of RF in twins is 20%
• Last 30 year mortality from RF is
decline gradually.
Cont..

• But in developing countries RF is still

causes mortality and disabilities.

• incidence of RF in developed countries is

decline.

• Antibiotic therapy decline incidence of RF

Pathology

• RF affected all body

• Mainly affected connective tissue

– Cardiovascular change
– Extra cardiac change
Cardiovascular changes

• Myocardium:
• (Aschoff nodule)→ Pathognomonic of RHD

• Myocardium or Subendocardium of LV

• Aschoff body persist for many years and sing of

Rheumatic chronic inflammation in RHD pts.

• Finally Aschoff body change to tringle scar and

placed in muscles and around vasculatures.
Aschoff nodule
Cont..

• Pericardium:
• Pericarditis
• Serofibrinous Pericardial effusion
• Both layer of pericard getting thick and
calcified, but
• no adhesion
Cont..

• Endocardium:
• Rheumatic endocarditis due to Verrucous
valvulitis of acute Rh. Fever

• Vegetation
– Placed on endocard and valve
– Cause mild valve regurgitation

• Causes thickening, fibrinous and

adhesion of commissura and chordea
tindinea
 Valve involvement in Acute RF

• Mitral (75 – 80%)

• Aorta (30%)
• Tricuspid (5%)
• Pulmonary (0.5%)
 Extra Cardiac manifestations

• Joints
– Swelling of Joints and soft tissues
– synovial layer red
– thick and cover with Fibrinous exudates
– More Proliferative than exudative
– no deformity
Extra Cardiac manifestations

• Subcutaneous nodules
– Presented in acute pahse, Granuloma
tissue, local edematous, subcutaneous
• Pleura
– Fibrinous pleurisy
• Lungs
– Pneumonitis, but never Aschoff body
• Chorea
– Normal CSF and protein
Clinical Manifestations

• Major Criteria:
• Polyarthritis
• Carditis
• Chorea
• Subcutaneous nodules
• Erythema Marginatum
 Arthritis

• Often involve big joints :

– (ankle, knee, Hip, wrest)
• Rare joints :
– (Sternoclavicular & Temporomandibular)
• Polyarthritis occur 60 – 75%
• Migratory
• Duration of inflammation is 1 – 5 week
• Response to Aspirin during 12 – 48 hour
Carditis

• Occur in 50 – 60%
• The first clinical finding
– MM of MR or AR
– In sever cases sign & symptom of:
• Pericardial effusion and CHF
– Death or sever deformity

•
Cont..

• Tachycardia
• Gallop rhythm
• Arrhythmia
• pericardial friction rub
• Heart block
• PR interval prolongation,
– due to Pericarditis:
• chest pain & friction rub
Cont..

• MM is indicator of carditis clinically:

• Endocarditis;
• (Mid diastolic MM (Carey-coomb’s MM) or
Systolic MM Grade III –VI)
– Early diastolic MM AR
– Holosystolic MM MR
– Rumbling diastolic MM MS
 Cont..

• Pericardial friction rub

• Pericardial effusion in Echo
• Chest-X-Ray (Cardiomegaly)
• Sign of Acute HF Acute phase
• Sign of Chronic HF Chronic phase
Subcutaneous nodule

• Incidence 5%
• Late manifestation 2-3 week after
incidence of Rheumatic fever

• Duration: few days to 3 week

• Size: 1-2cm without pain
• No adhesion with skin
Cont..

• Close to joints which is covered by less

muscles and tendons like:
– (patella, elbow joint, cranium, scapula,
Spinous process of vertebra and limbs)

• Recurrent nodules and Can’t be differentiate

from subcutaneous in Rheumatoid Arthritis
Subcutaneous nodule
Chorea

• Incidence is > 2-30% late manifestation

• CNS disorder
• Involuntary muscular movement without any
goal
– Appear in all body even in face and tongue
– Disappear by rest & appear by sever exercise
and excitement
– Generalized or Hemichorea
Continue

– Chorea without any sign of RF, pure chorea

– Chorea clinically started gradually
– At the beginning problem in writing, painting

– Incidence: more in female than male

– Duration: weeks to months
– Relapses: CNS stimulators
– Controlling: Sedative
Erythema Marginatum

• Red & pink eruption:

– equal or higher than skin level
• Ship: Round or irregular
• Trunk, Gluteal & lower limb (Never on face)
• Migratory
• Appears by heat & disappear by pressure
• No itching & thickness
Erythema Marginatum
Erythema nodosum

– Red & hard nodule

– More often in extensor area of the limbs
– Different size (cm)
– Painful & Sensitive by touching
– Pain getting sever by movement of limbs
• TB & Streptococcal pharyngeal
Rheumatic pneumonitis

• Rarely occur in RF
• Dx is clinically difficult, specially
when heart failure is present.
Minor Criteria

• Fever:
• 37.5 – 38.5C, rarely 39C
– With carditis or arthritis
– Continuously or intermittent
– Weakness, fatigue, anorexia and weight
lose

• Arthralgia:
– due to inflammation of tendon & muscles
Cont..

– Previous Rheumatic fever & RVHD

– ↑ ESR
– + CRP
– ECG PR interval prolongation
– Leukocytosis
– Abdominal pain due to peritonitis or liver
congestion
Cont..

– Tachycardia
– Epistaxis
– Pleuritic pain
– Arrhythmia
– Positive β-Hemolytic Streptococc in culture
Lab Exam
• Non of Lab. Exam is diagnostic of RF
• Antistreptolysin O (A.S.O)
• Adults >250 Todd unit
• Child more than 5year >333Todd unit
– Antideoxyribonuclease B (Anti DNase B)
– Antihyaluronidase (A.H)
– Antistreptozyme (ASTZ) > 200iu
– Culture form throat sowab is less
important than antibody test
– Leukocytosis
– ↑ Mucoprotein, Complement &
– ↑ in Alpha and Gama globulin
Cont..

• Anemia:
– due to decline of erythropoeisis
• Urine exam:
– Mild protienuria & Mic. Hematuaria
• ECG:
– PR interval prolongation (25%)
– Sometimes:
• Pericarditis ST elevation
• Myocarditis T – inversion
Chest – X – Ray

– Always normal
– Sometime after serial X-ray Cardiomegaly
– Marked cardiomegaly due to Pericardial
effusion

• Echocardiography:
– Diagnostic if pericardial effusion
– Not necessary in early stage
Prognosis
– Variable
– 75% RF 6 weeks
– 90% RF 12 weeks
– < 5% is more than 6 month
– Relapse of RF:
• in first 5 year after first attack often occur
– With aging gradually decline
– Relapse is due to:
• Incidence & Severity of strep, if RHD is present or no
and frequency of attacks.
Cont..

– Almost in 70% carditis 1st week

– 85% 12th week
– Carditis almost always occurs in all patient of
RF during 6 months
Rheumatic carditis & RVHD

• Variable due to:

– Duration & severity of inflammation
– Size of scar on valve & myocard
– Hemodynamic changes due to:
• Regurgitation and stenosis
– Incidence of frequent attack of carditis
– Calcification and sclerosis on valve
Diagnosis:

• 2 major creteria or
• 1 major and 2 minor creteria
• With positive finding for strep.
• But
– If clinical finding is not clear
– The Dx is difficult
DDx
• Infective Endocarditis:
– Fever
– Murmurs
– joint pain
– DDx: blood culture

• Gonococcal polyarthritis:
– Rapid response to penicillin
– Detection of infection in synovial fluid
DDx

• Rheumatoid arthritis:
– Small joints
– joint deformity
– positive R. factor

• Systemic lupus erythematosis

– Butterfly facial rash
– Elevated LE cell
– Involvement of kidney & pleura
Continue

• Congenital heart diseas:

– Angiography
– Cardiac Catheterization

• Drug sensitivity:
– Urticaria
– Angioneuritic edema

• Sickle cell anemia

– Joint pain, cardiomegaly, MM
DDx

• Chronic meningococcemia:
– blood culture
– clinical manifestation
• Osteomylitis:
– Lab exam
– bone radiography
• Lyme disease:
– Joint pain & fever
• Surgical Abdomen (Appendicitis)
Complications

• In serious case CHF

• RVHD
• Arrhythmia
• Pericarditis & Pericardial effusion
• Rheumatic Pneumonitis
• Pulmonary emboli
• Pulmonary infarction
 Treatment

• There is no specific treatment for RF

• No methods to modify attack of RF

• Conservative treatment decrease

complication & morbidity
General treatment

• Bed rest until:

– To normal the temperature without taking
medicine
– Pulse rate becomes < 100/min in rest
– Sed. Rate becomes normal
– To normalize ECG changes
Chemotherapy

• Penicillin for:
– Strep. Group A even culture is (-)
• Benzathin Penicillin
– 1200000 iu IM single dose
• Penicillin Procaine
– 600000 iu IM for 10 days.
Chemotherapy

• Salicylate:
– Dose increase gradually till sign of intoxication:
– Headach, tinitus and hyperpnea
– 100-125mg/kg/day for children
– 6-8 g/day for adults

• Naproxan 10-20mg/kg/day
Cont..

• Prednisone 60-120mg/day/QID
• For treatment of carditis some experts:
– Corticosteroid/Aspirin, but no positive data
– After DC:
• Sign of relapse, More side effects:
– Hirsutism, acne, cushingoid changes
– So treatment should start with aspirin, if not
effective change to steriod
Cont..

– After control of inflammation the drug should

continue till Sed. Rate become normal.
– Maintenance for weeks
– Steroid tapering in 2 weeks
– Aspirin continue 2-3 weeks after DC of Steroid
Cont..

• Rebound:
– Short in duration
• If mild, no need for course of anti
inflammatory drug
• Because after DC maybe rebound for 2nd
and 3rd time.
– The attack of RF in 5% long for 6 or
more month
Treatment of Chorea

– Complete mental & physical rest

– Salicylate & Corticosteroids are less effective

or not effective.

– Carbamazipine & Valproat sodium is better

than Haloperidol
Cont..

– If the other sign of RF is covered and

chorea is also start to cover, so the
patient can start daily tasks.

– No neurological deficit after chorea

– Intravenous Immunoglobulin also

effective
 Prevention of relapse

• Benzathin penicillin 1200000 iu monthly IM

• Oral Penicillin 200000 iu twice daily

• Pencillin sensitive patient

– Salfadiazin 1g daily
– Erythromycin 250mg twice daily

• Duration:
• Age < 18 life long
• RF without carditis age > 18 for 5 years