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)

Rheumatic Fever

PATHO

  • 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)

SSX

  • 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

DX

  • 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

TX

  • 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

Rheumatic Heart disease

PATHO

  • 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

  • Rheumatic fever - type 2 hypersensitivity

  • Rheumatic heart disease - type 4 hypersensitivity

  • Tricuspid is affected but generally the pt doesn't show up for this

  • MS can ➔ a-fib & ↑ thromboembolism risk

SSX

  • 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

DX

  • Anti-M antibodies

  • ECHO (Trans-thoracic) ➔ morphological features of RHD

    • Thickened anterior mitral valve leaflet

      • > 3mm in < 20yo, > 4mm in 21-40yo, > 5mm in >40yo

    • Chordal thickening

    • ↑ systolic mitral valve leaflet motion

    • Restricted leaflet motion (MV or AV)

    • Thickened AV

    • Coaptation defect of AV

    • AV prolapse

TX

  • 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

Prophylaxis

  • 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