Rheumatic fever Flashcards
Definition
systemic immune disease due to pharyngeal infection with group A beta-hemolytic streptococci involves the heart, joints, CNS, skin, subcutaneous tissue
classification - clinical variant (2) , clinical signs (major, minor),outcome (2), changes in heart, duration
According to clinical variant
- Acute
- Recurrent
According to clinical signs
a. Major manifestation
- Carditis
- Erythema marginatum
- Polyarthritis
- Subcutaneous nodules
- Chorea
b. Minor manifestations
- Fever
- Raised ESR or C-reactive protein
- Arthralgia
- Previous rheumatic fever
- Leukocytosis
- 1st or 2nd degree AV block (prolonged PR)
- Mitral/aortic regurgitation
- preceding streptococcal infection
According to outcome
- Recovery
- Chronic rheumatic heart disease with or without valve involvement
According to changes in heart
a. Active phase
- Rheumocarditis without valve disease
- Relapsing rheumocarditis (at places of old valve disorders)
- Rheumatic fever without heart changes
b. Non active phase
- Myocardiosclerosis
- Valve disorders
- Changes in other organs (chorea, nephritis, hepatitis, changes in skin, encephalitis, meningitis)
According to duration
- Acute –attack duration before 2 months, exudates, good effect from treatment
- Subacute – slow attack, 3-6 months, has exudation, no absolute effect from treatment
- Relapsing -attack acute (6-12 months), has exudation, not good effect from treatment
- Long – slow attack, (6-12 months), no exudation, some effects from treatment
- Latent –has valve disorders,
Etiology, pathogenesis - factors lead to strepto infection
Group A streptococci (streptococcal sore throat)- pharyngeal route
Virulence properties
1. serotypic surface M protein
2. hyaluronic acid capsules
pathogenesis
- Molecular mimicry‖ type of autoimmunity
- streptococcal antibodies higher in patients with acute rheumatic fever
- non-type-spesific peptides of M protein - cross-reactive immunologically with myosin, keratin, other coiled proteins found in cardiac tissues
- Hyaluronic acid of group A streptococcal capsules & hyaluronic acid of human host tissue - chemically identical
Factors that lead to Strepto attack:
1. Strepto infection
2. weak immunity
3. chronic (Strepto Ag ↑ by time to time)
4. Ag irritates humoral immunity
5. Sensibilization
Morphological stages of rheumatic disease
Stage 1 : Period of exudation -From time of infection till 2 months. Absent of clinical features. Difficult to diagnose.
- Stage 2 : Cells infiltration - 3-6 months. Develop clinical signs and features. Develop generalized inflammatory response.
- Stage 3 : Sclerotic organic changes - Deformation of organs. Forms granulomas by Aschoff Nodular.
Phases:
1. Mucoid swelling
2. Fibrinoid changes
3. Granulomatosis
4. Sclerosis
Dukket jones - major minor
Major manifestation
- Carditis
- Erythema marginatum
- Polyarthritis
- Subcutaneous nodules
- Chorea
- recent streptococcal infection
Minor manifestations
- Fever
- Raised ESR or C-reactive protein
- Arthralgia
- Previous rheumatic fever
- Leukocytosis
- 1st or 2nd degree AV block (prolonged PR)
- Echocardiography – mitral
- recent scarlet fever, raised antistreptolysin O or other streptococcal antibody titer, positive throat culture
Dukket jones manifestations
(2 major/1 major + 2 minor manifestations)
clinical picture
Sudden onset (2-3 weeks after acute pharyngeal streptococcal infection) with
1. Fever (38-39°C)
2. Sweating
3. Joints pain
4. Malaise
5. Loss of appetite
Peak - between ages 5-15, rare - before 4, after 40
- Rheumocarditis
- changes in endocardium (valve disorders) & myocardium
- Myocarditis
a. soft heart tones
b. enlarged heart borders
c. systolic murmur above mitral valve
d. gallop rhythm
e. heart insufficiency – dyspnea, fatigue, edema - Signs of rheumocarditis
a. Heart murmurs
b. Dilatation of heart chambers
c. heart failure
d. Pericardial effusion → pericarditis devmt
e. Inflmtory edema - Thickening of cusps & slight scarring
- Valves Firstly
a. formation of commissures (1st time)
b. 2nd time – dev incompetence (shrinking & coalescence)
c. Aortic & other valve D – after many recurrent attacks
- Arthritis
- In young ppl, polyarthritis, large joints (knees, elbow, ankles), visible charac of inflmtn (defiguration, edematous, skin pink & hot, pain (upon palpation or spontaneous)
- May dev rapidly & has migrating character, respond to treatmt
- In adult, normally is arthralgia - Chorea
- For young ppl
- Can‘t control mimic mm & extremities
- Hyperkinetic activity of joints
- Responds to treatmt - Skin changes – in young (allergy)
- Kidneys
- 2‘ glomerulonephritis
- (hematuria, proteinuria) - Liver – reactive hepatitis
- Changes in eyes
- Meningitis, encephalitis
investigations - throat-blood anal-biochem-urinalysis
- Throat swab culture for streptococcal infection
- Antibody tests for preceeding streptococcal infection:
1. Antistreptolysin O increased titers
2. antihyaluronidase
3. Antistreptozyme test - hemagglutination reaction to concentrate of extracellular streptococcal antigens absorbed to RBC - Raised ESR
- Increased C-reactive protein in serum
- Leukocytosis
- Increased mucoproteins, alpha-2, gamma globulins
- Anemia (suppression of erythropoeisis)
Biochemical-dysproteinemia, absent or decrease antistreptolysin
Urine analysis- decrease specific gravity, present red cells, leukocytes protein casts
D(x)
endocarditis
treatment - etio, pathogen
etiological
- streptococcus antibiotic - penicillin,amoxicillin - 2 weeks
- no long time antibiotic therapy
- 2-3 weeks injection LA antibiotics
pathogenic - antiinflammator - nsaid,corticosteroids
nsaid - orthrofan - 0,25mg 3x daily, buthodine,brofine,indomethacin, ibuprofen,ketoprofen
corticosteroids - glucocorti - prednisolone 20/30mg/day - very acute
prophylaxis and management
- Local streptococcal infection treatment e.g. pharyngitis and tonsillitis by AB minimum 5-7 days.
- Antibiotic therapy – long acting penicillin every 3 weeks during minimal 5 years
- Seasonal prophylaxis – spring + autumn when immunity is decreased- use anti inflmtory remedies for 1 mth to avoid inflmtory rXn - Annual prophylactic in 3-5 years
- If recurrent – can be long life antibiotic therapy
- NSAID – selective; non selective (aspirin, inhibitor SOX-II – meloxican)
- 5 years - rheumatic fever without carditis
- > 5 years (or all life) - rheumatic fever with carditis
- Management:
- Bed rest till CRP normal for 2 weeks- 3 months
- Analgesia for carditis/arthritis: aspirin for 6 weeks, monitor salicylates level. Alternative: NSAIDs
- Steroids improve symptoms
- Immobilize joints in severe arthritis
- Haloperidol or diazepam for chorea
- Secondary prophylaxis:
- Penicillin till no longer at risk. Alternative : suldadiazine. give antibiotics prophylaxis for dental and other surgery.
