Demam Rematik Dan Pjr AAS

8/13/2019 Demam Rematik Dan Pjr AAS

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DEMAM REMATIKdan

PENYAKIT JANTUNGREMATIK Abdullah Afif Siregar

Departemen Kardiologi dan Kedokteran VaskulerFakultas Kedokteran USU

Medan



Rheumatic fever is an immunologically mediatedinflammatory disease, that occurs as a delayedsequel to group A streptococcal throat infection,in genetically susceptible individuals.

Rheumatic heart disease is the most seriouscomplication of rheumatic fever

Acute rheumatic fever and rheumatic heartdisease are thought to result from anautoimmune response , but the exactpathogenesis remains unclear



• The rheumatic fever follows 0.3% of cases of group Abeta-hemolytic streptococcal pharyngitis in children.

• As many as 39% of patients with acute rheumatic fevermay develop varying degrees of pancarditis withassociated valve insufficiency, heart failure, pericarditis,

and even death.• With chronic rheumatic heart disease , patients develop

valve stenosis with varying degrees of regurgitation,atrial dilation, arrhythmias, and ventricular dysfunction.

• Chronic rheumatic heart disease remains the leadingcause of mitral valve stenosis and valve replacement inadults and children



Pathophysiology:• Rheumatic fever develops in children and adolescents following

pharyngitis with group A beta-hemolytic Streptococcus (ie,Streptococcus pyogenes ).

• The organisms attach to the epithelial cells of the upperrespiratory tract and produce a battery of enzymes allowing themto damage and invade human tissues.

• After an incubation period of 2-4 days, the invading organismselicit an acute inflammatory response with 3-5 days of sore throat,fever, malaise, headache, and an elevated leukocyte count.

• In 0.3-3% of cases, infection leads to rheumatic fever severalweeks after the sore throat has resolved . Only infections of thepharynx initiate or reactivate rheumatic fever.

• The organism spreads by direct contact with oral or respiratorysecretions , and spread is enhanced by crowded living conditions.





• The pathogenic mechanisms involved in the development of RF remainunclear . But it is evident that an abnormal humoral and cellular immuneresponse occurs.

• Antigenic mimicry between streptococcal antigens, mainly M-proteinepitopes and human tissues , such as heart valves, myosin andtropomyosin, brain proteins, synovial tissue and cartilage has beenproposed as the triggering factor leading to autoimmunity in individuals

with genetic predisposition.• Several genetic markers of susceptibility have been studied but no

consistent association found. Associations with different HLA class IIantigens have been observed in several populations.

• Molecular mimicry was first demonstrated by humoral immune response.

Streptococcal antibodies cross-react with several human tissues includingheart, skin, brain, glomerular basement membrane, striated and smoothmuscles .

• The presence of CD4+ T cells at lesions sites in the heart has beendemonstrated, suggesting a direct role of these cells in the pathogenesis ofRHD.

Etiopathogenesis :



Figure 1: Schematic representationof the aetiopathogenic events

occurring during the developmentof carditis

• Infiltrating T lymphocytes fromheart lesions of severe RHDpatients and peripheral Tlymphocytes were capable ofrecognising immunodominant

myocardium M5 peptides andvalve proteins . These resultsshowed the significance ofmolecular mimicry betweenbeta hemolytic streptococci and

heart tissue assessing the T-cellrepertoire leading to local tissuedamage in RHD .

Etiopathogenesis :



DIAGNOSIS :





Sambungan Tabel 4.1



Carditis (40% )



Clinical picture of carditis :• The clinical picture includes high pulse rate , congestive

heart failure, arrhytmias and pericardial friction rubs .• On the first attack, valvulitis is suspected in the presence

of a new apical systolic murmur of mitral regurgitation

(associated or not with an apical mid-diastolic murmur)and/or a basal diastolic murmur of aortic regurgitation.• Cardiomegaly is noted on X-Ray and on echocardiogram .• Myocarditis and/or pericarditis in the absence of valvular

involvement is unlikely due to acute RF

Carditis (40% )





Polyarthritis (75%)

• Arthritis is the most common manifestation,present in 60-80% of patients.

• It usually affects the peripheral large joints ;small joints and axial skeleton are rarelyinvolved.

•

Knees, ankles, elbows and wrists are the mostfrequently affected. The joints are red, warmand swollen.

• Arthritis is characteristically asymmetrical,migratory, and very painful , although somepatients may present mild joint complaints. Itusually resolves spontaneously at the most in2 or 3 weeks.

• Arthritis in ARF has an excellent response tosalicylates

http://www.netterimages.com/image/2507.htm




Sydenham Chorea :

Sydenham’s chorea is

characterized by involuntarymovements, specially of the faceand limbs, muscle weakness,disturbances of speech and gait.Children usually exhibitconcomitant psychologicdysfunction , especiallyobsessive-compulsive disorder,increased emotional lability,hyperactivity, irritablility andage-regressed behavior.It is usually a delayedmanifestation , and is often thesole manifestation of ARF.





Erythema marginatum : This is an evanescent, erythematous, non-pruritic rash withpale centers and rounded or serpiginous margins. Lesions

occur mainly on the trunk and proximal extremities andmay be induced by application of heat



Diagnosis :

Based on Jones Criteria, 1992 Update :- 2 Major criteria + 1 Minor criteria, or

- 1 Major criteria + 2 minor criteria* plus supporting evidence of preceding GAS

infection





Medical therapy involves the following 5 areas:1. Treat group A streptococcal infection regardless of organism

detection.2. Steroids and salicylates are useful in the control of pain andinflammation. The nonsteroidal anti-inflammatory drug (NSAID)naproxen has also been studied. It is effective and may be easierto use than aspirin.

3. Heart failure may require digitalis .4. Administer prophylaxis to patients who have developed ARF .

Patients with ARF should receive prophylaxis against futureGABHS infections. Available regimens include benzathinepenicillin G 1.2 million U IM every month, penicillin V 200,000 Uor 250 mg PO bid, or erythromycin 250 mg PO bid . Mostauthorities suggest that prophylaxis be given for 5 years. Forthose who have rheumatic carditis, some authorities suggest life-long prophylaxis.

5. Haloperidol may be helpful in controlling chorea.

Treatment :



Drug Name Penicillin G benzathine (Bicillin LA)

Penicillin G procaine (Crysticillin, Wycillin)

Penicillin VK (Beepen-VK,Betapen-VK, Robicillin VK, Veetids)

Description Interferes with synthesis ofcell wall mucopeptide duringactive multiplication resultingin bactericidal activity againstsusceptible bacteria.Because of its prolonged bloodlevel, several authors believethis to be the DOC. Othersprefer daily injections.

Long-acting parenteral penicillin(IM only) indicated in thetreatment of moderately severeinfections caused by penicillinG-sensitive microorganisms.Some prefer 10-d therapy.

Administer by deep IM injectiononly into the upper outerquadrant of the buttock.

Inhibits the biosynthesis of the cell-wall mucopeptide and is effectiveduring the stage of activemultiplication. Inadequateconcentrations may produce onlybacteriostatic effects. Penicillin VKis the oral alternative for thetreatment of rheumatic fever.

Adult Dose 2.4 million U IM once 2.4 million U IM once 500 mg PO q6h for 10 d

Pediatric Dose Infants and children <30lb: 600,000 U IM once

Children 30-60 lb: 900,000to 1.2 million U IM once

Infants and children <30 lb:600,000 U IM

Children 30-60 lb: 900,000to 1.2 million U IM

<12 years: 25-50 mg/kg/d POdivided tid/qid; not to exceed

3 g/d >12 years: Administer asin adults

Contraindications Documented hypersensitivity Documented hypersensitivity Documented hypersensitivity

Interactions Probenecid can increasepenicillin effectiveness bydecreasing its clearance;coadministration oftetracyclines can decrease

penicillin effectiveness

Increases risk of bleeding whenadministered concurrently withwarfarin; ethacrynic acid,aspirin, indomethacin, andfurosemide may compete with

penicillin G for renal tubularsecretion increasing penicillinserum concentrations

Probenecid may increaseeffectiveness by decreasingclearance; tetracyclines arebacteriostatic, causing a decreasein the effectiveness of penicillins

when administered concurrently Pregnancy B - Usually safe but benefits

must outweigh the risks.B - Usually safe but benefitsmust outweigh the risks.

B - Usually safe but benefits mustoutweigh the risks

Precautions Caution in impaired renalfunction

Never use IV route to administer penicillin G procaine; administer >10 d to eliminate organism and prevent complications.

Caution in renal impairment



Drug Name Erythromycin (EES, E-Mycin, Ery-Tab, Erythrocin)

Description DOC for patients allergic to penicillin; inhibits RNA-dependent proteinsynthesis, possibly by stimulating the dissociation of peptidyl t-RNAfrom ribosomes, which inhibits bacterial growth.In children, age, weight, and the severity of infection determine theproper dosage. When bid dosing is desired, one-half the daily dosemay be administered q12h. For more severe infections, the dose maybe doubled.

Adult Dose 1 g/d PO divided bid for 10 d

Pediatric Dose 30-50 mg/kg/d PO divided bid

Contraindications Documented hypersensitivity; hepatic impairment

Interactions Coadministration may increase toxicity of theophylline, digoxin,carbamazepine, and cyclosporine; may potentiate anticoagulanteffects of warfarin; coadministration with lovastatin and simvastatin,increases risk of rhabdomyolysis

Pregnancy B - Usually safe but benefits must outweigh the risks.

Precautions Caution in liver disease; estolate formulation may cause cholestatic jaundice; GI adverse effects are common (give doses pc);discontinue use if nausea, vomiting, malaise, abdominal colic, orfever occur



Drug Category: Glucocorticoids

Drug Name Prednisone (Deltasone, Sterapred)

Description

Patients with carditis require prednisone instead of aspirin. The goal is to

decrease myocardial inflammation.Useful in treatment of inflammatory and autoimmune disorders.Reversing increased capillary permeability and suppressing PMN activitymay decrease inflammation.

Adult Dose 60-80 mg/d PO

Pediatric Dose 2 mg/kg/d PO

Contraindications Documented hypersensitivity; viral, fungal, or tubercular skin infections

Interactions

Coadministration with estrogens may decrease clearance; concurrent usewith digoxin, may cause digitalis toxicity secondary to hypokalemia;phenobarbital, phenytoin, and rifampin may increase metabolism ofglucocorticoids (consider increasing maintenance dose); monitor forhypokalemia with coadministration of diuretics

Pregnancy B - Usually safe but benefits must outweigh the risks.

Precautions

Abrupt discontinuation of glucocorticoids may cause adrenal crisis;hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease,hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis,growth suppression, and infections may occur with glucocorticoid use



Anti inflammatory.

Drug Name Aspirin (Ascriptin, Bayer Buffered Aspirin, Ecotrin)

Description Treats mild to moderate pain. Inhibits prostaglandin synthesis, which preventsformation of platelet-aggregating thromboxane A2.

Adult Dose 6-8 g/d PO for 2 mo or until ESR has returned to normal

Pediatric Dose 80-100 mg/kg/d PO for 2 mo or until ESR has returned to normal

Contraindications Documented hypersensitivity; liver damage, hypoprothrombinemia, vitamin Kdeficiency, bleeding disorders, asthma; because of association with Reyesyndrome, do not use in children ( <16 y) with flu

Interactions

Effects may decrease with antacids and urinary alkalinizers; corticosteroidsdecrease salicylate serum levels; additive hypoprothrombinemic effects andincreased bleeding time may occur with coadministration of anticoagulants;may antagonize uricosuric effects of probenecid and increase toxicity ofphenytoin and valproic acid; doses > 2 g/d may potentiate glucose lowering

effect of sulfonylurea drugsPregnancy C - Safety for use during pregnancy has not been established.

Precautions

Pregnancy category D if full dose given during third trimester; may causetransient decrease in renal function and aggravate chronic kidney disease;avoid use in patients with severe anemia, with history of blood coagulationdefects, or taking anticoagulants



D g C t g I t i t



Drug Category: Ino t rop ic agen ts Some believe that digoxin may be helpful in congestive heart failure.

Drug Name Digoxin (Lanoxin)

Description

Cardiac glycoside with direct inotropic effects and indirect effects on the cardiovascular system.Effects on the myocardium involve a direct action on cardiac muscle that increases myocardial systoliccontractions and indirect actions that result in increased carotid sinus nerve activity and enhancedsympathetic withdrawal for any given increase in mean arterial pressure.

Adult Dose 0.125-0.375 mg PO qd

Pediatric Dose

Digitalizing dose:2-5 years: 30-40 mcg/kg PO5-10 years: 20-35 mcg/kg PO>10 years: 10-15 mcg/kg POMaintenance dose: 25-35% of PO loading dose

Contraindications Documented hypersensitivity; beriberi heart disease; idiopathic hypertrophic subaortic stenosis;constrictive pericarditis; carotid sinus syndrome

Interactions

Medications that may increase digoxin levels include alprazolam, benzodiazepines, bepridil, captopril,cyclosporine, propafenone, propantheline, quinidine, diltiazem, aminoglycosides, oral amiodarone,anticholinergics, diphenoxylate, erythromycin, felodipine, flecainide, hydroxychloroquine, itraconazole,nifedipine, omeprazole, quinine, ibuprofen, indomethacin, esmolol, tetracycline, tolbutamide, andverapamil; medications that may decrease serum digoxin levels include aminoglutethimide, antihistamines, cholestyramine, neomycin, penicillamine, aminoglycosides, oral colestipol, hydantoins,hypo

glycemic agents, antineoplastic treatment combinations (including carmustine, bleomycin, methotrexate, cytarabine, doxorubicin, cyclophosphamide, vincristine, procarbazine), aluminum or magnesiumantacids, rifampin, sucralfate, sulfasalazine, barbiturates, kaolin/pectin, and aminosalicylic acid

Pregnancy C - Safety for use during pregnancy has not been established.

Precautions

Hypokalemia may reduce positive inotropic effect of digitalis; IV calcium may produce arrhythmias ;hypercalcemia predisposes patient to digitalis toxicity, and hypocalcemia can make digoxin ineffective;magnesium replacement therapy must be instituted in patients with hypomagnesemia; patientsdiagnosed with incomplete AV block may progress to complete block when treated with digoxin;exercise caution in hypothyroidism, hypoxia, and acute myocarditis



Table : Secondary prevention of rheumatic fever.

Agent Therapeutic Scheme

Benzathine penicillin G 1,200,000 U every 4 weeks*, IMor

Penicillin V 250mg twice daily, POor

Sulfadiazine 500mg once daily for patients < 27kg; 1g once daily

for patients > 27kg, POFor individuals allergic to penicillin and sulfadiazine:

Erythromycin 250mg twice daily, PO

*In high-risk situations, administration every 3 weeks is recommended.



Table. Guidelines for Bed Rest and Ambulation andRecommended antiinflammatory agents

Arthritis Carditis Carditis Carditis

alone minimal moderate severe

Bed Rest 1-2 wk 2-3 wk 4-6 wk 2-4 mo

Indoor ambulation 1-2 wk 2-3 wk 4-6 wk 2-3 mo

Outdor activity 1-2 wk 2-3 wk 4-6 wk 2-3 mo(school)

Full activity 1-2 wk 2-3 wk 4-6 wk 2-3 mo

Prednisone 0 0 2-4 wk 2-6 wk Aspirin 0 0 2-4 wk 2-6 wk

M inimal Carditis Questionable cardiomegaly ; M oder ate car diti s definite but mild cardiomegaly ,

Severe carditis , marked cardiomegaly or CHF



ComplicationsCarditisMitral stenosisCongestive heart failure (CHF)

PrognosisSequelae are limited to the heart and aredependent upon the severity of the carditisduring the acute attack.

.



Rheumatic Heart Disease

Rheumatic heart disease is the most serious complicationof rheumatic fever.

Acute rheumatic fever follows 0.3% of cases of group Abeta-hemolytic streptococcal pharyngitis in children. Asmany as 39% of patients with acute rheumatic fevermay develop varying degrees of pancarditis withassociated valve insufficiency, heart failure, pericarditis,and even death. With chronic rheumatic heart disease , patients developvalve stenosis with varying degrees of regurgitation, atrial dilation, arrhythmias , and ventricular dysfunction .

Chronic rheumatic heart disease remains the leadingcause of mitral valve stenosis and valve replacement inadults



Frequency: In the US: Prevalence of rheumatic heart disease in the United States now is less than0.05 per 1000 populationInternationally: The incidence of rheumatic fever and rheumatic heart disease has notdecreased in developing countries. Retrospective studies reveal developingcountries to have the highest figures for cardiac involvement and recurrencerates of rheumatic fever. Estimations worldwide are that 5-30 million children

and young adults have chronic rheumatic heart disease, and 90,000 patientsdie from this disease each year. There were no data available in Indonesia



Mortality/Morbidity:Rheumatic heart disease is the major cause of morbidity fromrheumatic fever and the major cause of mitral insufficiency and

stenosis in the Indonesia and the world.Variables that correlate with severity of valve disease include thenumber of previous attacks of rheumatic fever, the length of timebetween the onset of disease and start of therapy, and sex. (Thedisease is more severe in females than in males.) Insufficiency fromacute rheumatic valve disease resolves in 60-80% of patients whoadhere to antibiotic prophylaxis.Race:The race (when controlled for socioeconomic variables) has not beendocumented to influence disease incidence.Sex: Rheumatic fever occurs in equal numbers in males and females, butthe prognosis is worse for females than for males.



Socio-economic factors :

It is well known that socioeconomic and environmentalfactors play an indirect, but important, role in the magnitudeand severity of RF and RHD. Such factor as a shortage ofresources for providing quality health care, inadequate

expertise of health-care providers, and a low level ofawareness of the disease in the community can all impactthe expression of the disease in populations. Crowdingadversely affects rheumatic fever incidence

Age:Rheumatic fever is principally a disease of childhood,

with a median age of 10 years, although it also occurs inadults (20% of cases).







Cardiac manifestations of chronic rheumatic heart disease : • Valve deformities,• thromboembolism,•

cardiac hemolytic anemia , and• atrial arrhythmias are the most common cardiac manifestations of chronicrheumatic heart disease.

Valve deformities • Mitral stenosis – Mitral regurgitasi occurs in 25% of patients

with chronic rheumatic heart disease and in association withmitral insufficiency in another 40%.

•

Aortic regurgitasi –

Aortic stenosis are typically from chronicrheumatic heart disease. The valve commissures and cuspsbecome adherent and fused, and the valve orifice becomes smallwith a round or triangular shape.



• Thromboembolism occurs as a complication of mitralstenosis. It is more likely to occur when the left atriumis dilated, cardiac output is decreased, and thepatient is in atrial fibrillation.

• Cardiac hemolytic anemia is related to disruption ofthe red blood cells by a deformed valve. Increaseddestruction and replacement of platelets also mayoccur.

• Atrial arrhythmias typically are related to a chronicallyenlarged left atrium (from a mitral valve abnormality).Successful cardioversion of atrial fibrillation to sinusrhythm is more likely to be successful if the left atriumis not markedly enlarged, the mitral stenosis is mild,and the patient has been in atrial fibrillation for lessthan 6 months.





Surgical Care:

When heart failure persists or worsens after aggressivemedical therapy for acute rheumatic heart disease,surgery to decrease valve insufficiency may be life-saving.Forty percent of patients with acute rheumatic fever

subsequently develop mitral stenosis as adults.In patients with critical stenosis, mitral valvulotomy,percutaneous balloon valvuloplasty, or mitral valvereplacement may be indicated.Due to high rates of recurrent symptoms after

annuloplasty or other repair procedures, valvereplacement appears to be the preferred surgical option.

TREATMENT





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Demam Rematik Dan Pjr AAS

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