RHEUMATIC FEVER

Collagen vascular disease with inflammation involving many organs mainly heart, brain, joints and skin.

The acute form of the disease follow infection with gp A β hemolytic streptococcus of the tonsillo-pharynx (only) with a latent period 2-4 wks.

The importance of Rf is its ability to cause fibrosis of the cardiac valves, leading to a devastating hemodynamic heart diseasesand regarded as the most common cause of heart disease in childhood and young adults world wide.

Incidence :• developed Countries decreased ,housing ,medical care• developing countries still high incidence(1/ 1000) age :6-15 yrsAffected population: militaries ,school contacts, m=fClimate: worm tropicalA β hemolytic streptococcus M strain epitopes shared with human heart ,myocin ,sarcolemal tissueAttack rate:3% in risky people

Symptoms: 1/3 mild sore throat 60% no historyThe problem is recurrence 50% vs. 3%Familiar clustering ,genetics ,HLA,

Pathology:

Fibrinoid degeneration

fragmentation all organs involvedcollagen

edema

infiltration,mononuclear histiocyte heart(IVS) aschoff cells

hyaline degeneration fibrosis calcification

Diagnosis There is no single specific clinical ,lab or test .Duckett jones criteria:

Major criteria:1. Carditis2. arthritis 3. Chorea4. Erythema marginatum5. Subcutaneous nodule Minor criteria: 1-clinical finding : arthritis Fever2-Lab investigation : raised acute phase reactant

CRP,ESR prolong PR intervalPrevious signs of streptococcal infection .Raised ASOT .Throat

culture

CARDITISPAN, PREVALENCE ~ 50%,MR ,ARTHE HALLMARK OF ARF

ArthritisMigratory, large , multi, asymmetrical, Inflammatory, salicylate responsive ,benignremain for 2-3wkPrevalence 70% ,

Sydenham ChoreaSt.vitus dance, chorea minorInvoluntary, purposeless, uncoordinated movement with weakness& emotional liability.basal ganglia, caudate nucleusAbout 20%,a later manifestation(3 months vs 3 wks)During awake ,hand writing , remain for 1-2 wksFor the future neuro psychosis, obsession ,recurrence

Erythema MarginatumEvanescent, erythematous, macular rash ,pale center , rounded edge, on the trunk and extremities sparing the face . More rare 5%,More clear by applying heat

Subcutaneous nodule

Fine , painless ,freely mobile,nodule,0.5-2cm,on the extensor surface of joints occipital &spinal processRare 3%, often associated with carditis

Minor criteriaFever and joint pain are non specific , for support diagnosis if had single major criteria.Lab investigation:• acute phase reactant raised early, normal in chorea• ECG prolong PR interval

Previous signs of streptococcal infection:ASOTThroat culture

Treatment(RASSA) RestSalicylate SteroidAntibiotics: Primary prevention Secondary prevention