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8/16/2019 Slide Sindrom Behcet RYP
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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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