Demam Rematik Dr.didik

DEMAM REMATIK

Masrul SyafriBag Kardiologi dan Kedokteran vaskuler

FKUA / RS Dr M Jamil

04/12/231

Epidemiologi Etiologi / faktor risiko Patogenesis Manifestasi klinis Prinsip diagnosis Penatalaksanaan

04/12/232

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

04/12/233

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

04/12/234

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

04/12/235

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)

04/12/236

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

9

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

04/12/2312

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

04/12/2313

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

04/12/2314

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

04/12/2316

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

04/12/2319

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

04/12/2320

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)

04/12/2321

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

04/12/2322

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

04/12/2330

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

04/12/2331

Jones criteria (1944)Jones criteria (1944)

Jones modification (1955)Jones modification (1955)

Jones revision (1965)Jones revision (1965)

Update (1992)Update (1992) (2003)

04/12/2332

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

04/12/2337

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

04/12/2339

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