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SINDROM

BEHÇETRani Yunita Pat

PEMBIMBING

Prof. DR. dr. ST. RUKIAH SY

dr. &UNAEDI SIRA&UDD

dr. ST. SORAYA TAU'IK"

Dr. HASNAH" S#.M


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OverviewEpidemiologiAnatomiEtiologiPatogenesisGejalaDiagnosisDiagnosis banding TerapiKomplikasiPrognosis


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Pendahuluan

•

indrom !eh"et adalah pein$amasi kronis% sering kamerupakan suatu oklusi vassistemik dengan etiologi #ang diketahui&

(Triple SymptomComplex (


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ejarah


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EP)DE*)O+OG)

•

,The ilk -oute disease.

Prevalensi indrom !eh"et

1& /0 '00 kasus per 100&000 pendudu Turki

2& /10 per 100&000 di epang

'& 0%3 per 100&000 di Amerika erikat&


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EP)DE*)O+OG)

•

lakilaki 5 wanita

• 24 dan '4 tahun

• 6amilial dan sporadik


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EMBRIO!OGI TRAKU*EA

8E9-A+ E:TODE-*


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A8ATO*) T-AKT9 9


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1=& Khurana AK& Anatom#%Ph#siolog# and Diseases o> The E#e% )n ? :omprehensive Ophthalmolog# )nternational Publishers&8ew Delhi&200;2=& +awren@e *&+ dkk& The E#e% )n ? !asi@ and :lini@al @ien@e :ourse e@tion 2% 6undamentals and Prin

Ameri@an A@adem# o> Ophthalmolog#& an 6ransis@o& 2013

• !agian paling anterior dari

traktus uvea• *emberikan warna pada mata melanosit dari selsel pigmen

• truktur iris tersusun ataspembuluh darah dan jaringanpen#ambung

• Diameter iris sekitar 12 mmdengan keliling antara '; '/mm dan ketebalan 0%4 mm

IRIS


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KORPUS SI!IARIS• !erbentuk se

potongan sagmm C 7%4 mtemporal dapada nasal =&

• Apeksn#a posterior mora serata

• !agian berbatasan diris dan mesklera melaotot longitumasuk ke skl


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KOROID !agian traktus uve

posterior terbentan

optik sampai ora se Ketebalann#a 0%24

atas ' lapisan korpaling dalam% pebagian tengah dbesar bagian luar

Permukaann#a d

melekat erat pada permukaan luarn#apada sara> optik siliaris posterior dmemasuki bola matkeluarn#a vena vort


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• 9vea ban#ak mengandung komponen

dari sistem imun termasuk makro>ag% selim>osit%neutroFl dan sel plasma

• Respon Imun Infamasi

Si)t(+ I+un Tra,tu)U-(a


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Si)t(+ I+un Ada#tif


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ET)O+OG)

•

!elum diketahui

Human Leucocyte

Antigen $H!A%/0


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PATOGE8E)

• Agen Infeksius• Mekanisme Imun

•

Faktor Genetik


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Agen Infeksius

•

erpes simple virus tipe 1• epatitis : virus

• Streptococcus sanguis

•Mycobacterium tuberculosis


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Mekanisme Imun

Autoi+un

• -esponse to immunosuppressive medi@atio

• Presen@e o> T l#mpho@#tes in lesions

• Th1 proFle o> @#tokines released >rom blooC)68γ % )+2% )+12=

• )mmunogeneti@ asso@iations

• Autorea@tive T @ells Ceg antihsp70=


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Faktor Genetik

Kelompok ras tertentu

Hresikoindrom !eh"et

+A!4 >enotipe dan subtipe n#a

!w41+AD-1 dan +ADIwl J menurunkan resiko indrom !e


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GEA+A K+)8)


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Gejala Klinis Okular


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Gejala Klinis 8on Okul


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D)AG8O)

Diagnosis indrom !eh"et berdasdari temuan klinis dan kriteriadiagnostik& Terdapat dua sistemdiagnostik% #aitu ?

istemistem


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istem Diagnostik epan


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istem diagnostik )nternational tud# Grou!eh@et Disease


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D)AG8O) !A8D)8G

•

9veitis anterior• indrom artritis reakti>

• ar@oidosis

•

istemik lupus erithematosus• egener granulomatosis

• 8e@rotiLing retinitis herpes


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TE-AP)

•

Korti,o)t(roid• I+uno+odu1ator

aLathioprine% in$iimab%

@#@losporine% ta@rolimus%m#@ophenolate mo>etil% kloramatau siklo>os>amid&


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KO*P+)KA)

Katarak:#stoid makula edema C:*E=

Glaukoma neovaskularisasi

Perdarahan vitreous


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P-OG8O)

• ampir 24M dari pasien di seluruh dunia d

indrom !eh"et o@ular kronis memiliki ketvisual kurang dari 20N200% paling seringdisebabkan oleh edema makula% vaskulitisoklusi% atroF optik% dan glaukoma&

• i>at kekambuhan kronis pada pen#akit indengan sering eksaserbasi setelah lama rmembuatn#a sulit untuk memprediksi hasvisual&


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Penutup• indrom !eh"et adalah suatu pen#akit kronis%

berulang% gangguan in$amasi multisistem% terditandai dengan trias klasik berulang #aituperadangan okular% lesi kulit% dan ulkus oral dagenital #ang berulang& Pen#akit ini sering melisendi% P% dan saluran pen@ernaan&

• Agen in>eksius% mekanisme imun% dan >aktor g#ang terlibat dalam etiopatogenesis pen#akit masih harus diselidiki&

• Diagnosis indrom !eh"et tergantung identiFk

beberapa kombinasi Ftur klinis #ang khas&


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Penutup• Pengobatan adalah masalah #ang rumit

• Kombinasi kortikosteroid dan imunomodulato

• Komplikasi seperti edema makula% katarak #akompleks% glaukoma% glaukoma sekunder danglaukoma neovaskular% retina dan optik disku

neovaskularisasi% ablasi retina% dan perdarahvitreus sangat mempengaruhi visual prognosPrognosis indrom !eh"et baik jika dilakukandiagnosis dan pengobatan se@ara dini&


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TERIMA KASIH


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• R(a2ti-( art3riti) is @lassiFed as an

autoimmune @ondition that develops inresponse to an in>e@tion in another partbod# C@rossrea@tivit#=& :oming into @onwith ba@teria and developing an in>e@tio

trigger the disease• +A 2;

• :onjun@tivitis dan uveitis

https://en.wikipedia.org/wiki/Autoimmune_diseasehttps://en.wikipedia.org/wiki/Autoimmune_disease


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• Sar2oido)i)% also @alled )ar2oid% is a

disease involving abnormal @olle@tionsin$ammator# @ells that >orm lumps knogranulomas&1 The disease usuall# begthe lungs% skin% or l#mph nodes& +ess

@ommonl# aQe@ted are the e#es% liver%and brain&

• +A D-

https://en.wikipedia.org/wiki/Granulomashttps://en.wikipedia.org/wiki/Sarcoidosishttps://en.wikipedia.org/wiki/Sarcoidosishttps://en.wikipedia.org/wiki/Granulomas


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• S4)t(+i2 1u#u) (r4t3(+ato)o>ten abbreviated as S!E or 1u#a s#stemi@ autoimmune diseaseautoimmune

@onne@tive tissue disease= in whthe bod#Rs immune s#stem mistakenl# atta@ks health# tissu

https://en.wikipedia.org/wiki/Systemic_autoimmune_diseasehttps://en.wikipedia.org/wiki/Autoimmunityhttps://en.wikipedia.org/wiki/Connective_tissue_diseasehttps://en.wikipedia.org/wiki/Immune_systemhttps://en.wikipedia.org/wiki/Immune_systemhttps://en.wikipedia.org/wiki/Connective_tissue_diseasehttps://en.wikipedia.org/wiki/Autoimmunityhttps://en.wikipedia.org/wiki/Systemic_autoimmune_disease


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• Granu1o+ato)i) 5it3 #o14angiiti) CGPA=%

re>erred to as (g(n(r6) granu1o+ato)i) a s#stemi@ disorder that involves bothgranulomatosis and pol#angiitis& )t is a >orm ovas@ulitis Cin$ammation o> blood vessels= thasmall and mediumsiLe vessels in man# orga

• E#es? pseudotumours% s@leritis% @onjun@tivitisepis@leritis

• Antineutrophil @#toplasmi@ antibodies CA8:A

https://en.wikipedia.org/wiki/Granulomahttps://en.wiktionary.org/wiki/polyangiitishttps://en.wikipedia.org/wiki/Vasculitishttps://en.wikipedia.org/wiki/Scleritishttps://en.wikipedia.org/wiki/Conjunctivitishttps://en.wikipedia.org/wiki/Episcleritishttps://en.wikipedia.org/wiki/Anti-neutrophil_cytoplasmic_antibodyhttps://en.wikipedia.org/wiki/Anti-neutrophil_cytoplasmic_antibodyhttps://en.wikipedia.org/wiki/Anti-neutrophil_cytoplasmic_antibodyhttps://en.wikipedia.org/wiki/Anti-neutrophil_cytoplasmic_antibodyhttps://en.wikipedia.org/wiki/Anti-neutrophil_cytoplasmic_antibodyhttps://en.wikipedia.org/wiki/Anti-neutrophil_cytoplasmic_antibodyhttps://en.wikipedia.org/wiki/Anti-neutrophil_cytoplasmic_antibodyhttps://en.wikipedia.org/wiki/Episcleritishttps://en.wikipedia.org/wiki/Conjunctivitishttps://en.wikipedia.org/wiki/Scleritishttps://en.wikipedia.org/wiki/Vasculitishttps://en.wiktionary.org/wiki/polyangiitishttps://en.wikipedia.org/wiki/Granuloma


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• A-8 and PO-8 represent a spe@trumrapidl# progressing ne@rotiLing herperetinopathies& )mmuno@ompetent hodevelop peripheral retinitis a@@ompa

b# vas@ulitis% irido@#@litis% and vitritis• A@ute retinal ne@rosis CA-8= and

progressive outer retinal ne@rosis CPO


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• #stemi@ lupus er#thematosus other vas@uliti@ s#ndromes musruled out& Patients with !eh@ets#ndrome have negative antinu

and antineutrophili@ @#toplasmiantibodies&


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