RHEUMATIC HD

= an inflammatory disease of connective tissues involving mainly joints heart CNS (less frequent) Etiology - both initial and recurrent attacks are non-suppurative, sequelae to URTI with group A beta hemolytic streptococcal (GABS) - high tendency to recur

Epidemiology Attack rate Recurrence rate Familial susceptibility Socio economic condition

Age Sex

follows 3% of untreated/inadequately treated attacks of URTI with GABS attack rate increase to 50% of URTI with GABS in pt with past hx of RF 10-20% of pt have +ve family hx overcrowding, poor sanitation, poverty common in 3rd world countries more during fall ,winter & early spring 5-15 yrs rare <3 yrs girls = boys

Prerequisites to develop RF - A rheumatogenic strain of GABS (Strains that produces rheumatic fever - M types l, 3, 5, 6,18 & 24). - A susceptible host. - Overcrowding which favors easy and rapid spread of GABS - Occurrence of URTI, not skin - Persistence of infection untreated for more than one week. Pathogenesis (Mechanism is not definite) 1- Abnormal immune response to GABS - a genetically predisposed person reacts abnormally to a component of GABS. - reaction takes 1-3 weeks - Ag/Ab complexes formed cause immunological damage to the CT of heart & joints Pathologic Lesions 1- Acute Exudative stage This stage lasts for 2-3 weeks - HEART: interstitial cellular infiltrate (lymphocytes & plasma cells) + edema of the valvular ring & leaflets + fibrinous or serofibrinous pericarditis - JOINTS: infiltration of synovial membrane + serous effusion swelling of the articular and periarticular structures 2- Similarity of streptococcal & human Ags molecular mimicry streptococcal Ag immunologically similar to human tissue Ag elicits Ab capable of reacting not only with the microbial products, but also with the hosts Ag.

2- Proliferative stage Fibrinoid degeneration of connective tissue results in formation of Ashcoff nodules - limited to myocardium & endocardium Final results; - Pancarditis in the heart - Arthritis in the joints - Ashcoff nodules in the SC tissue - Basal ganglia lesions chorea

Diagnosis: Rheumatic fever is mainly a clinical diagnosis No single diagnostic sign or specific laboratory test available for diagnosis Diagnosis is based on; MODIFIED JONES CRITERIA Jones Criteria (Revised) for Guidance in the Diagnosis of Rheumatic Fever* Major Manifestation 1. Carditis 2. Polyarthritis 3. Chorea 4. Erythema Marginatum 5. Subcutaneous Nodules Clinical Minor Manifestations Laboratory 1. Acute phase reactants : - ESR - C-reactive protein - leukocytosis 2. Prolonged P-R interval Supporting Evidence of Streptococal Infection - Recent Scarlet Fever - Rapid Ag test - Increased Titer of Anti-Streptococcal Antibodies ASO (anti-streptolysin O) - +ve Throat Culture for GABS

1. Previous rheumatic fever or rheumatic heart disease 2. Arthralgia 3. Fever

high probability of acute rheumatic fever if there are; 2 MAJOR criteria + evidence of preceding GABS infection Or 1 MAJOR + 2 MINOR criteria + evidence of preceding GABS infection Except in the following 3 situations ; - Chorea alone (isolated), if other causes have been excluded - Insidious or late-onset carditis with no other explanation - Rheumatic recurrence = Patients with documented RHD or prior rheumatic fever, presence of 1 MAJOR criterion OR of fever/arthralgia/high CRP with evidence of preceding GABS infection Clinical pictures: - RF develops in 3 phasese; Phase 1 GABS infection of URT in the form of pharyngitis/tonsillitis, may be; - moderately severe/very mild/asymptomatic

Phase 2 symptoms of URTI subsides followed by a latent period (1-3 wks), during which pt is symptoms free

Phase 3 acute onset of RF manifestations - always associated with fever (except in isolated chorea) * sometimes subacute esp in chorea & late onset carditis Evidence of preceding GABS infection of URT 1. Hx of scarlet fever - by 1wk to 1 month 2. +ve throat culture 3. +ve rapid test for streptococcal Ag 4. elevated ASO titer = >350 Todds unit in children > 5 y/o - best demonstrated in between acute & convalescent serum 5. Pt suspected with RF but NO in ASO tested for other strep. Ab (anti DNAse)

Minor Jones Criteria = arthralgia + fever + acute phase reactant + prolonged PR interval acute phase reactant Arthralgia High ESR Elevated C-reactive protein - pain in the joint in absence - normal : up to 10mm/hr - normal : up to 6mg/l of signs of inflammation (warmth,swelling,redness) simple & reliable, but - tends to disappear in the - pain must be in the joint - may be normal in = convalescent stage before (not in the muscle/ HF, chorea, erythema ESR returns to normal periarticular tissue) marginatum, old standing - often absent in chorea, - must be distinguish from nodules eryhtme marginatum and nocturnal pain in - occasional in = pt taking long standing nodules extremeties that is normal in long acting penicillin - remains high when CHF children - returns to normal within complicates carditis - not used as minor criterion weeks /months depending if polyarthritis is included as on severity major criterion used to monitor disease activity

MAJOR JONES CRITERIA Migratory polyarthritis

50% of cases

Rheumatic Pancarditis (Carditis)

Sydenham Chorea
- 10-20% of cases - girls - occur much later than other manifestations (several months after the attack of RF) - clinically manifest as i) emotional instability ii) chorea iii) hypotonia & weakness of voluntary muscle iv) incoordination (clumsiness)

Erythema Marginatum
- uncommon - occur in <5% of cases - lasts for few days - pink, serpiginous macules with pale centres - not itchy - more on trunks & limbs

Subcutaneous nodules
- rare - only 1% of cases - lasts for 1-4 months - short-like, firm, non tender bodies - mainly over extensor surfaces of joints elbow, knee, knuckles, ankles, spine, scapulae & scalp

- affects 40-70% of pt with acute RF within the 1st 3 weeks of attack - most frequent in the younger age group - Severity of carditis is inversely related to severity of arthritis - red, warm, swollen, - manifest as pancarditis very tender & fleeting endocardium murmurs of valve lesions - migratory polyarthritis - mitral aortic regurge - limited movements + myocardium - cardiac enlargement, tachycardia, gallop, arrhythmia effusion pericardium pericardial rub or effusion - involving large joints Endocarditis : (knee, ankle, elbow & Acute phase Chronic phase wrist) valvulitis fibrosis, calcification & - rare toes & fingers stenosis of heart valves (fishmouth valves) . Acute stage murmurs Chronic stage murmur Murmur is due to incomplete closure of the valve, dt Mitral insufficiency Aortic insufficiency Mitral stenosis : dilatation of mitral valvular (regurgitation) (regurgitation) fibrous ring pan systolic murmur early diastolic mid diastolic rumbling : edema of valve leaflet or - beast heard : apex murmur develop about 5 yrs after chorda tendinae propagation : axilla soft blowing acute attack of rheumatic : papillary ms dysfx S1 : muffled - best heard : left carditis dt scarring of the rd th - dt lengthened conduction 3 , 4 space valve time occur early in the During diastole, mitral leaflets S2 : accentuated 1st attack float on top of the blood - dt relative diminution of S1 - usually with MR filling the LV when systole (rarely alone) starts, the valve is already half-closed making the + mitral diastolic murmur closure less forcible (Carey comb/inflow) st valvular component of 1 HS - in some chidren with mitral insufficiency during acute stage of RF - soft apical - dt BF from LA to LV = relative MS * sould be diff. from rumbling murmur of MS - disappears within 12- the ONLY manifestation of RF that give CHRONICITY & permanent damage to the 24 h after salicylate organ (chronic valve disease- rheumatic HD) - NO chronicity, leaves - can be mild (lasting for few weeks) /severe (lasting up to 6 months)/ the joint intact rarely fulminant causing death

Clinical signs - pronator sign - milking sign

- elicited by local application of heat

- strong seropositivity - presence indicates severe carditis (both are proliferative lesion)

Management 1. Treatment of Group A B-Hemolytic Streptococci infection Penicillin - IM benzathine penicillin (long acting penicillin) once, OR - oral penicillin V, 10 days Penicillin-allergic patient - erythromycin, 10 days, OR - amoxicillin, 10 days, OR - cefadroxil, 10 days, OR - azythromycin, 3 days

2. Anti-inflammatory (suppressive) therapy Arthritis only Aspirin - blood level should be maintained at 20-25 mg/dl - duration : 2 weeks after return of ESR to near normal value Arthritis + Carditis Prednisone - until ESR return to near normal value - is then gradually withdrawn in 2-4 weeks (tapering dose) Aspirin - given from the start of prednisone tapering to overlap it + continued for 2-3 weeks after comple withdrawal of prednisone

3. Rx of rheumatic chorea *steroids & salicylates are NOT beneficial in isolated chorea - Haloperidol - Diazepam 4. Other supportive treatment Bed rest No carditis strict bed rest is NOT needed Carditis withOUT CHF keep in bed until ESR approaches normal Carditis + CHF bed rest until CHF is controlled & ESR approaches normal Congestive cardiac failure mild CHF complete bed rest + oxygen + fluid restoration + prednisone severe CHF same above + diuretics + small dose digitalis + K chloride 5. Prevention of RF or its recurrence 1ry Prophylaxis EARLY + ADEQUATE Rx of GABS infections * strep can be eradicated + RF can be prevented if penicillin is given: - early : within 7 days of GABS infection - in sufficient period of time : 10 days 2ry prophylaxis (Prevention of Recurrent Attacks) - IM Benzathine penicillin G every 2 weeks - Oral Penicillin V twice daily - Oral Sulfadiazine once daily * allergic pt to penicillin & sulfadiazine = oral Erythromycin twice daily

Duration of Secondary Rheumatic Fever Prophylaxis Rheumatic fever without carditis Rheumatic fever with carditis but no residual heart disease (no valvar disease*) Rheumatic fever with carditis and residual heart disease (persistent valvar disease*) *Clinical or echocardiographic evidence

5 y or until age 21 y, whichever is longer 10 y or until 21 y, whichever is longer At least 10 y since last episode and at least until age 40 y, sometimes lifelong

Prognosis - Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection, if not on prophylactic medicines - Good prognosis for older age group & if no carditis during the initial attack - Bad prognosis for younger children & those with carditis with valvar lesions