1Rheumatic fever

2 Content

Disease overall view Precipitating factors Diagnosis Management

3 Disease overall view

Rheumatic fever is a systemic autoimmune disorder related to prior streptococcal infection

The development of rheumatic fever is preceded by the upper respiratory tract infection (tonsillitis or pharyngitis) with Group A β-hemolytic streptococci.

4 Disease overall view

Autoimmune mediated by: Antigenic similarity: Abs formed against streptococcal Ags react with native human tissue antigens(autoimmune tissue damage)

The latent period between streptococcal infection & onset of RF is 1-5 wks.

5 Precipitating factors

Age: between 5-15 yrs.

Sex: equal except for rheumatic chorea (common in females).

Socio-economic factors: common in developing countries &low social classes (overcrowding &bad hygiene).

Recurrent and severe recent strept. infections

6 Diagnosis

Jone’s criteria Polyarthritis: w/ low grade fever, large

joints, migratory - often 1 at a time, No permanent dysfunction. (>75%)

Carditis, pericarditis, cardiomegaly, or valvulitis (the most serious manifestation & often results in permanent damage). (~50%)

Major

criteri

a

plus Evidence of a recent group A Strep infection

7 Diagnosis

Jone’s criteria Chorea: late occurrence, 1- 6 months

after the infection, self-limiting, resolves in 1- 3 months. (~30%)

plus Evidence of a recent group A Strep infection

Major

criteri

a

8 Diagnosis

Jone’s criteria Erythema Marginatum:

“classic” truncal rash, migratory - appears & disappears within hours.

(pink rash – irregular red edges serpiginous borders– clear center) (~10%)

plus Evidence of a recent group A Strep infection

Major

criteri

a

9 Diagnosis

Jone’s criteria Subcutaneous Nodules:

Late occurrence ( months after infection) Painless small nodules over bony prominences:ElbowsKneesspine. (1- 2%) plus

Evidence of a recent group A Strep infection

Major

criteri

a

10 Diagnosis

Jone’s criteria Fever Arthralgia (mild pain without objective

findings): Can only be considered without finding of arthritis

Elevated acute-phase reactants: ESR, C-reactive protein.

plus Evidence of a recent group A Strep infection

Minor

criteri

a

11 Diagnosis

Anti streptococcal Abs are investigated:ASOAnti-deoxy-ribonuclease (DNAse) BAnti-hyaluronidase

Heart reactive antibodiesTropomyosin is elevated in acute rheumatic

fever.plus Evidence of a recent group A Strep infection

Work

ups

12 Diagnosis

Diagnosis with rheumatic fever if you meet two major criteria, or one major and two minor criteria, and have signs that you've

had a previous strep infection.

Work ups

Minor

criteri

a

Major criteria

13 Complications

Early complications:( Heart failure, Arrhythmias)

Late complications:(Rheumatic valvular lesions, rheumatic

activity)

14

Management

Acute state

Prevent further attacks

2ry 1ry

15 Management

Prevention 1ry prevention by treating Strep.

Pharyngitis:The penicillin is the drug of choice because:

Narrow spectrum of activityLong standing efficacyLow cost

16 Management

Prevention IM benzathine penicillin:

1. 0.6 Millions Unit IM 1 Time (< 27 Kg)2. 1.2 Millions units IM 1 Time (>27 Kg)

1ry preventio

n

17 Management

Prevention PO - Phenoxymethyl Penicillin

(Penicillin V)1. Children (50mg/kg/day) 250 mg (2-3

times/day) x10 days2. Adolescent /Adult 500 mg TID x10

days10 days course therapy

(++time & --

compliance)

1ry preventio

n

18 Management

Adverse effect of penicillins Allergic reaction:

Anaphylaxis, UrticariaAngioedema, rash.

GIT: Diarrhea, vomiting & oral thrush

1ry preventio

n

19 Management

Adverse effect of penicillins Renal:

Acute interstitial nephritisCNS:

Convulsion & fever

1ry preventio

n

20 Management

Adverse effect of penicillins Site of injection:

Pain with IM route, inflammation at IV-site

Blood:Hemolytic anemia, thrombocytopenia, neutropenia

1ry preventio

n

21 Management

In case of penicillin allergy use:Oral erythromycin:(20-40 mg/kg/day)(2-4 times/day)(up to 1gm/day) for 10 days.Newer macrolidesOral cephalosporins

10 days course therapy

(++time & --

compliance)1ry preventio

n

22 Management 1ry preventio

n

23 Management

Antibiotics “NOT” Recommended for Strep. Pharyngitis:Sulfonamides, Trimethoprim,

Sulfamethoxazole (not eradicate GAS).Tetracyclines / Doxycycline / Minocycline.(High prevalence of resistant strain)

1ry preventio

n

24 Management

2ry prevention starts after diagnosis of RF: Rheumatic Fever requires “continuous”

prophylactic antibiotics due to:

1. Increased “susceptibility” to recurrences2. Increased “severity” of recurrences3. “Asymptomatic nature” of Strep. Infections.

2ry preventio

n

25 Management 2ry preventio

n

Or Whichever is longer

26 Management Acute phase

Treatment of:ArthritisCarditisChorea

27 Management Acute phase

Treatment of:Arthritis

Relieves by aspirin within 24-48 hrs of pain

The diagnosis is questionable if aspirin isn’t effective

Mild arthritis may respond to paracetamol alone

28 Management Acute phase

Treatment of:Arthritis

Aspirin dose for pediatrics:90-130 mg/kg/day in equally divided doses every 4-6 hrs, up to 5.5 gm/day.

29 Management Acute phase

Treatment of:Arthritis

Salicylate serum levels (20-25 mg/mL)After 1-2 wks, the dosage is decreased to 60-70 mg/kg/day and given for 1-6 wks or longer if necessary, then gradually withdrawn over 1-2 wks.

30 Management Acute phase

Treatment of:Arthritis

Naproxen/ Ibuprofen/ paracetamol are potential alternative

31 Management Acute phase

Treatment of:Carditis

Avoid strenuous exercise Treat symptoms of heart failureSteroids are preferred over salicylates Treatment is guided according to clinical status of the patient

32 Management Acute phase

Treatment of:Carditis === severe case< use prednisolone

2 mg/kg /day 2-4 divided doses (max 60mgm /day ) 2-3 wks [ ESR < 30mm / 1hr ] Taper off 5 mg / 3 days Add - Aspirin 75 mgm / kg / day x 8 -10 weeks

33 Management Acute phase

Clinical Severity Arthralgia or mild

arthritis; no carditis

Mod./severe arthritis; no carditis OR carditis without failure

Treatment Analgesics only

Aspirin 100 mg/kg/d with meals for 3 wk (or longer up to 6 wk); then taper gradually over 6 wk

34 Management Acute phase

Clinical Severity Carditis with

failure; with or without joint manifestation

Treatment Prednisone 40-60

mg/d, if necessary; after 3 wk, slow tapering over 3 wk, continue aspirin for 4 wk after d/c of prednisone.

35 Management Acute phase

Treatment of:Rheumatic chorea

Haloperidol : initial dose 0.5 to 1 mg and 0.5 is added every 3 days for Maximal effect or until a maximal dose of 5 mg/ day

Sodium valproate: (15 to 20 mg/kg per day) is also effective.

36 Management Acute phase

Treatment of:Rheumatic chorea

Diazipam 0.25-0.5 mg/Kg/day

37 Management Acute phase

Treatment of:Rheumatic chorea

In resistant cases, plasmaphersis iv Immunoglubulins.

Steroid therapy is generally not effective in Sydenham’s chorea.

38

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