Infectious - Inflammatory

Rheumatic Fever can ➔ Rheumatic heart disease

Acquired heart disease MC in children and young adults from a "strep throat" infection (can be skin too but LC)

GAS - Group A β-hemolytic Streptococcal (streptococcus pyogenes) infxn ➔ pharyngitis
2-4 weeks later ➔ inflammation rxn
- Acute pancarditis
  - Over years/multiple episodes can ➔ valvular disease

Inflammation rxn = non-suppurative (not pus) sequela (condition)
THEY THINK:
- GAS pharyngeal infxn ➔ AB against GAS antigen (M protein)
- Mimicry (M protein looks like nerve/myocardial proteins) ➔ cross-reactivity and TYPE 2 hypersensitivity rxn (cytotoxic)

JONES
- Joint - Migratory/additive poly-arthritis
- 🖤 Carditis - (usually endocarditis in form or valvulitis)
- Nodules - Firm, < 2cm, over bony prominence/tendons, elbow MC
- Erythema marginatum - Non-pruritic, pink annular rash
- Sydenham chorea - involuntary, non-rhythmic & non-repetitive movement (limbs, neck, face)

Immune complexes land in joints ➔ arthritis
- Migratory/additive: Starts in a joint, then starts in another. Seems to migrate/add to other joints
T cells bombing (infiltration) and AB binding to myosin ➔ pancarditis
Skin manifestation (Erythema marginatum/nodules) probably means carditis is present
- Erythema marginatum - usually in a ring with sharp outer edge, center returns to normal first (extends centrifugally)
AB bind basal ganglia ➔ chorea
- Sydenham chorea can also ➔ flailing (ballismus), muscle weakness, odd behavior

ASO titer
Jones criteria
- Recent hx of GAS infxn (throat culture, rapid strep, ↑ ASO or ADB) AND:
- 2 Major criteria: (Joint, 🖤, nodules, erythema marginatum, sydenham chorea)
- OR 1 major + 2 minor:
  - Polyarthralgia
  - Fever (> 101.3/38.5)
  - ↑ESR, CRP (acute phase reactants)
  - Prolonged PR
- Hx of rheumatic fever: 2 major, 1 major + 2 minor, 3 minor

Arthralgia is a symptom: joint pain
Arthritis is a sign (and a dx): intra-articular inflammation ➔ swelling/hot/erythema, ↓ROM, tenderness

β-lactam - PO penicillin V (phenoxymethylpenicillin)
- Macrolide (erythromycin/azithromycin) if PCN allergy
ASA for fever & joint pain, corticosteroids if refractory to NSAIDs
- Ibuprofen/naproxen for children
Culture swabs, rapid streps on the household

2-3 wks after GAS infxn ➔ pancarditis ➔ valvulitis (mitral > aortic) or third degree block
- Acute ➔ mitral/aortic regurgitation ➔ HF (in severe MR/AR),
- Over time ➔ MS/MR, AS/AR
Pt with valve problem may show up with a hx of rheumatic fever or there was a missed rheumatic fever dx
- So... just because they never "had" RF doesn't mean it isn't RHD

Mitral Stenosis sx
- DOE, hemoptysis, hoarseness
- MS MD rumble open snap (Mid Diastolic rumble with an opening snap)

Watch out for RHD in pregnant pts
- New demand on stenotic valve can ➔ worsening of sx

Treat HF (from the carditis) and refer to valve repair specialist to see what they want to do
- Emergent valve repair/replacement usually only needed if severe

IM penicillin G benzathine every 28d for:
- Rheum fever only ➔ 5 years or until 21yo (whichever is longer)
- HF and carditis but no valvular disease ➔ 10 years or until 21yo (whichever is longer)
- Rheum fever and valve disease ➔ 10 years or until 40yo (whichever is longer)
If GAS infxn develops despite prophylactic β-lactam: clindamycin

Next: Congenital Heart Dz

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