demam rematik dr.didik
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DEMAM REMATIK
Masrul SyafriBag Kardiologi dan Kedokteran vaskuler
FKUA / RS Dr M Jamil
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Epidemiologi Etiologi / faktor risiko Patogenesis Manifestasi klinis Prinsip diagnosis Penatalaksanaan
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DR, komplikasi lambat infeksi pharing oleh grup A streptokokus β-hemolitikus
Negara maju sudah berkurang sejak 1950
Penelitian terakhir (Jakarta), insiden DR dan PJR usia 5-15 tahun 0,3-0,8 per 1000 anak
RS M Djamil Padang (2000-2006) 60 kasus DR dan PJR
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Di AS terdapat peningkatan insiden , th 1997 menimbulkan kematian 5014 orang
RS Sardjito Yogyakarta, 25 DR dan PJR baru / tahun
Prevalensi tinggi pada daerah padat / sosio-ekonomi rendah / negara sedang berkembang
Worldwide estimated > 470,000 cases occur annually , approximately 340,000 cases in children 5-14 years of age
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Di RSCM (unit Anak) 60-80 kasus / tahun
Dipengaruhi faktor ras dan etnik, suku Maori di New Zealand lebih tinggi dibandingkan suku Kaukasoid setempat
Kembar (monozigot) 7x lebih sering dibandingkan dizigot
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Streptococcus beta haemolyticus grup A
Serotipe tertentu : M1,3,5,6,18 dan 24
Penelitian epidemiologi sebagian kecil (2-3%) penderita faringitis streptokokus menjadi DR
Serangan ulangan pada DR : 50% (rentan)
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Faktor risiko / predisposisi• Herediter / ras DR4 ras Caucasian, DR2 African-American population , HLA-DR3 India
• Umur ( usia ekolah )• Keadaan sosial ekonomi• Musim ( ISPA )/ winter and springtropical no consistant seasonal pattern
• Serangan terdahulu
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Faringitis strept β haemo A
Membentuk antibodi dalam serum
AB bereaksi dgn komponen protein otot jantung/valvula
Radang miokard (miokarditis)Valvulitis
perikarditis
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Kelainan histopatologis : sel Aschoff (Aschoff bodies) yang menetap
Edema interstitial Komponen sel Aschoff: monosit dan
makrofag yang berfungsi sbg antigen presenting cells berperan dalam karditis
Respon imunologi yang abnormal terhadap infeksi streptokokus (auto imun)
Defisiensi sistim imun host faktor predisposisi berkembangnya aktifitas rematik 04/12/23
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Konsep keserupaan antigenik, antibodi yang dihasilkan oleh infeksi streptokokus bereaksi silang dengan jaringan hospes (otot jantung).
Hipotesis terakhir : kerusakan jaringan adalah mekanisme imunologik humoral dan selular
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Parts of the organ affectedParts of the organ affected
• inflammatory lesions inflammatory lesions • swelling swelling Aschoff bodiesAschoff bodies• necrosis necrosis
It is not seen within 1st week after onset RFIt is not seen within 1st week after onset RFDont corelated with diseases activityDont corelated with diseases activity
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Kumpulan gejala dan tanda klinis (sindrom)
Bisa muncul bersaman atau sendiri (khorea)
Dapat mengenai multi organ (jantung, sendi, otak dan jaringan kutan /subkutan
Jantung karditis sampai gagal jantung
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Karditis• Sering (50-75%) pada Demam rematik akut• Penyebab paling serius / mortalitas tinggi• Dapat muncul sendiri atau bersama dengan
klinis lain• Dapat muncul belakangan, setelah artritis /
2-3 minggu serangan• Takikardia murupakan tanda klinis awal
suatu miokarditis
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Tanda klinis karditis rematik :• Bising patologis terutama bising regurgitasi
mitral• Pada MR berat dapat dijumpai bising
stenosis relatif di apeks (bising mid-akhir diastolik = carey coombs)
• Kardiomegali secara radiologis• Gagal jantung (karditis berat)• Tanda perikarditis (sesak dan nyeri dada,
pulsasi jantung lemah, pericardial friction rub)
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Insufisiensi aorta • Lebih kurang 20% pada karditis rematik• Bising diastolik awal dengan nada tinggi• Kebocoran yang berat kadang teraba thrill
diastolik • Nadi perifer melompat-lompat
(waterhammer pulse) tekanan sistolik yang sangat tinggi akibat kebocoran katup aorta
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Gagal jantung• Akibat insufisiensi katup yang berat• Terjadi sekitar 5% penderita DRA• Akibat datang pada keadaan serangan
ulangan / klinis berat sesak jika aktifitas (dispnea), orthopnea (sesak waktu tiduran), anoreksia, takikardia, kardiomegali, hepatomegali, dll.
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Merupakan klinis paling sering Tanda nyeri, bengkak, merah dan
panas Berat seperti pseudoparalisis
(gerakan sendi sangat terbatas) Pada umumnya mengenai sendi
besar (lutut, perbelangan kaki, siku, pergelangan tangan)
Tidak sama dengan antralgia
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Sendi-sendi kecil/perifer jarang terkena
Khas : asimetris dan berpindah-pindah (poliartritis migran)
Sebagian besar sembuh dalam 1 minggu
Respon sangat baik dengan salisilat Secara radiologis tidak ditemukan
kelainan
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Khorea minor / St. Vance dance Sekitar 15% pada penderita DR Keterlibatan sistem saraf sentral
terutama ganglia basal / nuklei kaudati Periode laten lebih lama ( sekitar 3 bln ) Gerakan-gerakan yang tidak
terkoordinasi Lebih nyata pada penderita bangun dan
stres (emosi yang labil)
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Kelabilan emosi tergambar dari, mudah menangis, tidak kooperatif, gelisah, mudah menunjukan reaksi yang tidak sesuai
Gejala menghilang 1-2 minggu, pada kasus yang berat bisa sampai 3-4 minggu
Dua kali lebih sering pada perempuan Jarang terjadi setelah pubertas, dan
tidak pernah terjadi pada dewasa
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Ruam kulit yang khas pada DR Tidak gatal, makular dengan tepi
eritema, diameter ± 2,5 cm Paling sering ditemukan di batang
tubuh dan tungkai proksimal Timbul sewaktu-waktu, tersering
pada stadium awal Sering menyertai karditis
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Frekuensi < 5% pada DR Biasanya pada permukaan ekstensor
sendi, ruas jari, lutut dan sendi kaki, kadang-kadang di kepala
Ukuran bervariasi dari 0,5-2 cm, tidak nyeri, dan dapat digerakkan secara bebas
Muncul pada minggu pertama sakit dan lebih cepat menghilang
Pada umumnya pada penderita dgn karditis
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NODUL SUBKUTAN
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Table 3. Combination of major manifestations RF/RHD Table 3. Combination of major manifestations RF/RHD patients patients at the Department of Child Health FKUI/CM Hospitalat the Department of Child Health FKUI/CM Hospital
Major manifestationMajor manifestation 1983-19871983-1987 1988-19921988-1992 1998-20001998-2000Arthritis onlyArthritis only 44 (15.2%)44 (15.2%) 69 (26.5%)69 (26.5%) 00Carditis onlyCarditis only 133 (45.9%)133 (45.9%) 144 (44.4%)144 (44.4%) 6 (27.3%)6 (27.3%)Chorea onlyChorea only 13 (4.5%)13 (4.5%) 5 (1.9%)5 (1.9%) 00Subcutaneous noduleSubcutaneous nodule 00 00 1 (4.5%)1 (4.5%)Erythema marginatumErythema marginatum 00 00 00Arthritis + carditisArthritis + carditis 73 (25.2%)73 (25.2%) 51 (19.91%)51 (19.91%) 13(59.1%)13(59.1%)Arthritis + choreaArthritis + chorea 8 (2.8%)8 (2.8%) 3 (1.2%)3 (1.2%) 00Arthritis + eryth. marginat. 2 (0.7%)Arthritis + eryth. marginat. 2 (0.7%) 4 (1.6%)4 (1.6%) 00Carditis + choreaCarditis + chorea 9 (3.1%)9 (3.1%) 3 (1.2%)3 (1.2%) 1 (4.5%)1 (4.5%)Carditis + subcutan. nodule 1 (0.3%)Carditis + subcutan. nodule 1 (0.3%) 6 (2.3%)6 (2.3%) 00Chorea + subcutan. nodule 1 (0.3%)Chorea + subcutan. nodule 1 (0.3%) 1 (0.4%)1 (0.4%) 00Chorea + eryth. marginat. 1 (0.3%)Chorea + eryth. marginat. 1 (0.3%) 00 00Arthritis + carditis + choreaArthritis + carditis + chorea 5 (1.7%)5 (1.7%) 2 (0.8%)2 (0.8%) 1 (4.5%)1 (4.5%)Total casesTotal cases 290290 257257 2222
Tidak spesifik Jarang melebihi 39’C Sering menyertai poliartritis dan
karditis Pada banyak kasus sembuh
sendiri dalam 2-3 minggu Tidak pernah pada khorea
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Nyeri sendi tanpa disertai tanda peradangan
Sering mengenai sendi-sendi basar
Pada nyeri hebat, kadang tungkai sukar digerakkan
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Positive throat culture or rapid antigent test ( rate of recovery is low )
ASTO 3-4 weeks following infection. 80-85% will have elevated titers
Elevated acute-phase reactants : CRP and erythrosite sedimentation rate
Minor criteria
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Jones criteria (1944)Jones criteria (1944)
Jones modification (1955)Jones modification (1955)
Jones revision (1965)Jones revision (1965)
Update (1992)Update (1992) (2003)
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Major manifestation Minor manifestation
Carditis Clinical finding Polyarthritis Arthralgia Chorea Fever Erythema marginatum Laboratory findings Subcutaneous nodule Elevated acute phase reactants
Erythrocyte sedimentation rate C-reactive protein
Prolonged PR interval
Plus supporting evidence of preceding streptococcal infection: ASO or
other streptococcal antibodies; positive throat culture; recent scarlet fever.
The presence of 2 major / 1 major + 2 minor manifestations high probability of ARF, except: chorea / long term carditis.
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Table . Guidelines for the diagnosis of Rheumatic fever Table . Guidelines for the diagnosis of Rheumatic fever according to Jones criteria, 1992 update. according to Jones criteria, 1992 update.
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Katagori diagnostik
Kriteria
Episode pertama DR. 2 mayor atau 1 mayor dan 2 minor + bukti infeksi streptokokus grup A sebelumnya.
Serangan ulang DR tanpa PJR. 2 mayor atau 1 mayor dan 2 minor + bukti infeksi streptokokus grup A sebelumnya.
Serangan ulang DR dengan PJR. 2 minor + bukti infeksi streptokokus grup A sebelumnya.
Reumatik korea.Reumatik karditis yang tiba-tiba.
Manifestasi mayor lainnya atau bukti infeksi streptokokus grup A tidak diperlukan.
Lesi katup kronis pada PJR (datang dengan murni gejala mitral stenosis atau kombinasi kelainan katup mitral dan/atau kelainan katup aorta.
Untuk diagnosis tidak memerlukan kriteria lain karena telah menunjukkan gejala PJR.
Kriteria WHO 2002-2003 untuk diagnosis DR dan PJR
WHO Technical Report Series. Geneva, 29 Oktober-1 November 2001.
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Management RF/RHDManagement RF/RHD
• bed restbed rest• eradicationeradication• anti inflammationanti inflammation• supporting therapy supporting therapy • secondary prophylaxis secondary prophylaxis • educationeducation• intervention / surgeryintervention / surgery
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Table : Management of RF/RHD (1)Table : Management of RF/RHD (1)
Clinical manifestation Bed rest Anti inflammatory drugsClinical manifestation Bed rest Anti inflammatory drugs
No carditisNo carditis Total: 2 weeks Total: 2 weeks Salicylates 100 mg/kg/ Salicylates 100 mg/kg/ gradual ambulation: day for 2 weeks & 75 gradual ambulation: day for 2 weeks & 75 2 weeks2 weeks mg/kg/day for 4-6 weeks mg/kg/day for 4-6 weeks
Carditis, no cardio-Carditis, no cardio- Total: 4 weeks Total: 4 weeksmegaly megaly gradual ambulation: gradual ambulation: same above same above
4 weeks4 weeksCarditis with cardio- Total: 6 weeksCarditis with cardio- Total: 6 weeks Prednisone 2 mg/kg/day Prednisone 2 mg/kg/daymegaly megaly gradual ambulation: for 2 weeks & taper of gradual ambulation: for 2 weeks & taper of
6 weeks 2 weeks; salicylates 756 weeks 2 weeks; salicylates 75 mg/kg/day at 2 weeksmg/kg/day at 2 weeks and continue for 6 weeksand continue for 6 weeks
Carditis, with heart Total: for as long asCarditis, with heart Total: for as long asfailure heart failure is present same abovefailure heart failure is present same above
gradual ambulation: for gradual ambulation: for 3 months3 months
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Table 4. Management of RF/RHD (2)Table 4. Management of RF/RHD (2)Clinical manifest. Eradication Sec. prophylaxis ActivityClinical manifest. Eradication Sec. prophylaxis Activity
No carditisNo carditis Benzathine pe- Benz. pen. 1.2 M School after Benzathine pe- Benz. pen. 1.2 M School after nicillin 1.2 M IM every 4 wks 4 wks hosp.nicillin 1.2 M IM every 4 wks 4 wks hosp. IU IM for 5 yrsIU IM for 5 yrs Sports: free Sports: free
Carditis, noCarditis, no same above Benz. pen. 1.2 M School after same above Benz. pen. 1.2 M School aftercardiomegaly cardiomegaly IM every 4 wks 12 wks hosp. IM every 4 wks 12 wks hosp.
until 18until 18thth yrs old Sports: free yrs old Sports: free (min. 5 yrs)(min. 5 yrs)
Carditis with same above Benz. pen. 1.2 M School afterCarditis with same above Benz. pen. 1.2 M School aftercardiomegalycardiomegaly IM every 4 wks 12 wks hosp. IM every 4 wks 12 wks hosp.
until 25until 25thth yrs yrs Sports: com- Sports: com- (min. 5 yrs)(min. 5 yrs) petitive sports petitive sports
prohibited. prohibited.Carditis withCarditis with same above Benz. pen. 1.2 M School after same above Benz. pen. 1.2 M School afterheart failureheart failure IM every 3 wks 12 wks CHF IM every 3 wks 12 wks CHF
until 25until 25thth yrs old cured yrs old cured (min. 5 yrs) Sports: prohibited(min. 5 yrs) Sports: prohibited
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Management of RF/RHDManagement of RF/RHD
Bed rest Anti inflammatory Eradication Secondary Bed rest Anti inflammatory Eradication Secondary drugsdrugs prophylaxis prophylaxis
Hospitali- Special precaution: Secondary Other alternative: Hospitali- Special precaution: Secondary Other alternative: zation during -gastritiszation during -gastritis alternative Penicillin allergy alternative Penicillin allergytotal bed rest -hypertensiontotal bed rest -hypertension Oral: penicillin sulpha: Oral: penicillin sulpha:
-tuberculosis -tuberculosis 4 X 500 mg 4 X 500 mg 12 yrs: 2X ½ tab.12 yrs: 2X ½ tab. (10 days) (10 days) 12 yrs: 2 X 1 tab.12 yrs: 2 X 1 tab.
Sulpha allergy:Sulpha allergy: erithromycierithromycicinecine 2 X 250 mg2 X 250 mg
Benz Pen G Once only
Reduce physical and emotional sterss Anti-inflamntory agents are not
indicated in patient with isolated chorea
For severe case : phenobarbital 15 – 30 mg every 6 to 8 hours ; haloperidol, valproic acid, CPZ, diazepam or steroid
Plasma excange (to remove antineural antibodies)
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Complete bed rest Restriction of sodium and fluid intake Digoxin (inotropics agent) Furosemide (diuretics) etc
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Supporting therapy :Supporting therapy : - high calory/protein intake- high calory/protein intake- roborantia- roborantia
Medication failedMedication failed intervention/surgery intervention/surgery
Secondary prophylaxis with benzathine penicillinSecondary prophylaxis with benzathine penicillin- 3 weeks/ 4 weeks interval - 3 weeks/ 4 weeks interval controversy ? controversy ?- Lue : 3 weeks interval- Lue : 3 weeks interval- Ayoub: 3 weeks interval in special cases- Ayoub: 3 weeks interval in special cases
Communication, information & Communication, information & educationeducation
Advanced casesAdvanced cases maintenance therapy of chronic heart maintenance therapy of chronic heart
failurefailure intervention (BMV, BAV)intervention (BMV, BAV) surgery (valve repair/replacement)surgery (valve repair/replacement)
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Step I - primary prevention (eradication of
streptococci) Step II - anti inflammatory treatment
(aspirin,steroids) Step III- supportive management &
management of complications
Step IV- secondary prevention (prevention of recurrent
attacks)
STEP I: Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis)
Agent Dose Mode Duration
Benzathine penicillin G 600 000 U for patients Intramuscular Once
27 kg (60 lb) 1 200 000 U for patients >27 kg
or Penicillin V Children: 250 mg 2-3 times daily Oral 10 d (phenoxymethyl penicillin) Adolescents and adults:
500 mg 2-3 times daily
For individuals allergic to penicillin
Erythromycin: 20-40 mg/kg/d 2-4 times daily Oral 10 d Estolate (maximum 1 g/d)
or Ethylsuccinate 40 mg/kg/d 2-4 times daily Oral 10 d
(maximum 1 g/d)
Arthritis only Aspirin 75-100mg/kg/day,give as 4divided doses for 6weeks(Attain a blood level 20-30 mg/dl)
Carditis Prednisolone 2-2.5mg/kg/day, give as twodivided doses for 2weeksTaper over 2 weeks &while tapering addAspirin 75 mg/kg/dayfor 2 weeks.Continue aspirin alone100 mg/kg/day foranother 4 weeks
Step II: Anti inflammatory treatmentClinical condition Drugs
Bed rest Treatment of congestive cardiac
failure: -digitalis,diuretics Treatment of chorea:
-diazepam or haloperidol Rest to joints & supportive splinting
3.Step III: Supportive management & management of complications
STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks)
Agent Dose Mode
Benzathine penicillin G 1 200 000 U every 4 weeks* Intramuscular
orPenicillin V 250 mg twice daily Oral
orSulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral
1.0 g once daily for patients >27 kg (60 lb)
For individuals allergic to penicillin and sulfadiazine
Erythromycin 250 mg twice daily Oral
*In high-risk situations, administration every 3 weeks is justified and recommended
Duration of Secondary Rheumatic Fever Prophylaxis
Category Duration
Rheumatic fever with carditis and At least 10 y since last residual heart disease episode and at least until (persistent valvar disease*) age 40 y, sometimes lifelong
prophylaxis
Rheumatic fever with carditis 10 y or well into adulthood, but no residual heart disease whichever is longer (no valvar disease*)
Rheumatic fever without carditis 5 y or until age 21 y, whichever is longer
*Clinical or echocardiographic evidence.
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