Sindrome di Sjögren - Altervistaoculistaweb.altervista.org/6nov/Franch.pdf · Ocular Surface...
Transcript of Sindrome di Sjögren - Altervistaoculistaweb.altervista.org/6nov/Franch.pdf · Ocular Surface...
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Sindrome di Sjögren
Antonella FranchU.O. Oculistica
Ospedale SS Giovanni e Paolo - Venezia
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Definizione Dry Eye
• Disordine del film lacrimale per deficit lacrimale o eccessiva evaporazazione che causa danno alla superficie oculare interpalpebrale ed è associata ad importanti sintomi di discomfort oculare
NEI/Industry Global Definition (Lemp 1995)
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Superficie oculare
Unità funzionale:
• Cornea
• Congiuntiva
• Ghiandole Meibomio
• Ghiandole lacrimali accessorie
• Ghiandola lacrimale principale
• Innervazione
• Film lacrimale
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Film lacrimale
strato esterno lipidico (ghiandole di Meibomio), strato acquoso (ghiandola lacrimale principale, e accessorie di Krause e Wolfring) e strato interno mucoso (cellule caliciformi mucipare)
recentemente rivista come una struttura a due strati: uno spesso strato muco-acquoso, in cui la concentrazione di mucina decresce dalla superficie della cornea in direzione dello strato più esterno, lipidico
mucine nella fase acquosa riducono la tensione superficiale e consentono al film di bagnare la cornea in modo uniforme, permettendo allo strato lipidico di distendersi e rallentare l’evaporazione delle lacrime
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The Ocular Surface System
LacrimalGlands
SecretomotorNerve Impulses
Tears Support and MaintainOcular Surface
Ocular SurfaceNeural Stimulation
Epithelial cell differentiation
and movement
Hormones
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Studi epidemiologici
• Salisbury Eye Evaluation
• Beaver Dam Eye Study
• Melbourne Visual Impairment Project
• Women’s Health Study
• Physicians’Health Study
• Blue Mountains Eye Study
• Shihpai Eye Study
• Sumatra Indonesia Study
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Descrizione dei sintomi
• 640 pazienti con dry eye
• 389 risposte diverse
• Peggioramento sintomi verso la fine della giornata
Begley CG et Al, Cornea 2002
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Sintomi più frequenti
• Secchezza 98,7%
• Affaticamento oculare 85,1%
• Sensazione di corpo estraneo 78,5%
• Rossore 71,6%
• Bruciore 64,8%
Nichols KK et Al, Optom Vis Sci 1999
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Studi epidemiologici
• Women’s Health Study
• Physician’s Health Study
• Alta Prevalenza 15-20% > 50aa
• Legata all’età (40000 donne >49aa - 27000 medici>50aa)
• Più frequente nelle donne (probabilità doppia)
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Dry Eye
Deficit produzione
Sr. SjögrenSr. non Sjögren
Aumento evaporazione
Meiboniangland disease
EsposizioneLAC/Blink
abnormality
Sr. Sjögren
Sr. Sjögrenprimaria
Sr. Sjögrensecondaria
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Non Sjögren Dry Eye
Non Sjögren
Lacrimal disease
Lacrimal Obstrution
Reflexblock
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Sindrome di Sjögren
• Disordine autoimmune
• Colpisce Gh. lacrimali e salivari
• Deficit produzione lacrime e secchezza delle fauci
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Sindrome di Sjögren
• Disordine autoimmune
• Fatt. rischio HLA-B8/DR
• Agente scatenante non noto
• Cell. Gh. lacrimali come APC
• Frammenti di cell. autoantigene
• CD4+ attivazione T cell/CD8
• Distruzione delle cellule epiteliali lacrimali
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Stimolo scatenante
• Infettivo (virus Epstein Barr/citomegalovirus)
• Processo immunitario (stress/apoptosi)
• Difetto epiteliale salivare
• Difetto innervazione (bassa attività recettore alfa 7 dell’acetilcolina di tipo nicotinico che aumenta n. macrofagi)
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Anticorpi
• Ro/SS-A, La/SS-B
• ICA 69
• a-b fodrina
• M3R (tipo 3 recettore muscarinico)
• Anticorpi multipli
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Classificazione American European Consensus Group Sr. Sjögren (rivista 2002)
I 1 su 3 Secchezza oculare >3mesi
Ricorrente sensazione di sabbia/CE
Uso di sostituti lacrimali>3 volte/die
II 1 su 3 Secchezza fauci >3mesi
Gonfiore ricorrente gh salivari
Necessità di bere per deglutire
III Test Schirmer + occhi chiusi/no anest
Test Rosa Bengala +
IV Positività biopsia gh salivare minore
V 1 su 3 Scialometria basale
Scintigrafia salivare
Scialografia
VI Positività anticorpi anti SSA/SSB
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Classificazione Sr. Sjögren secondaria
Presenza di altra malattia del connettivo ben definita
(AR, LES, Sclerodermia, polimiosite, poliarterite nodosa, tiroidite di
Hashimoto, Waldestrom, Raynaud, dermatomiosite, cirrosi
epatobiliare)
Positività ad almeno 1 della tab I e II
Positività ad almeno 2 della tab III, IV, V
Anamnesi: per assunzione farmaci interferenti secrezione lacrimale
(ansiolitici, antidepressivi, b-bloccanti, diuretici, antistaminici,
antiparkinsoniani, anticolinergici, antiipertensivi, analgesici,
antipsicotici)
Vitali C et Al, Ann Rheum 2002
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Workup Sr. Sjögren
OCULARE
Schirmer test
Es. LAF coloranti vitali
Es. fluorescina e BUT
ORALE
Es. denti
Biopsia gh. salivare minore
Scintigrafia salivare e scialometria
SISTEMICO
Consulenza reumatologica
VES,
anti-SSA,
anti-SSB,
FR, ANA,
IgG, IgM, IgA,
Criglobulinemia
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Workup Sr. Sjögren
DIAGNOSI
•Anamnesi
•Ispezione
•Esame alla LAF (Fluorescina, Fluorescina con filtro giallo, Rosa Bengala, Verde di Lissamina), BUT, NIBUT
•Frequenza ammiccamento
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DIAGNOSI
Ispezione
Esame alla LAF
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DIAGNOSI
Ispezione
Esame alla LAF
BLEFARITE!
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COLORANTI
FLUORESCINA altamente idrofilo (colora l’acqua), colorazione non bloccata dalla mucina
Strisce di carta bibuladiluizione 0,5-1 e 2%
Studio del film lacrimale, BUT, Turnover lacrimale
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COLORANTI
FLUORESCINA colora se degenerazione/morte cellulare
STAINING/POOLING/DIFFUSION
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Fluoresceina sodica: valutazione integrità epiteli di superficie
Colorazione diffusa, inferiore, interpalpebrale, superiore
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Fluoresceina sodica: menisco lacrimale
Valutazione dellelesioni corneali(ricerca del segno di Seidel)
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COLORANTI
FLUORESCINA con filtro giallo
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COLORANTI
FLUORESCINA con filtro giallo
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COLORANTI
ROSA BENGALA gtt 1%
Fototossico, usare anestetico?
colorabilità epiteliale inibita da albumina, mucine, carbossimetilcellulosa
Colora muco, cellule morte o degenerate
Tossico
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COLORANTI
VERDE DI LISSAMINA
Cartine 1,5 mg da bagnare con fisiologica
Colora cellule epiteliali con membrana cellulare danneggiata, non bloccato dalle mucine
Non tossico
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Verde di lissamina
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Workup Sr. Sjögren
TEST LACRIMALI
Test di Schirmer:
I: a palpebre aperte
Ib: a palpebre chiuse
Test di Jones: dopo instillazione anestetico topico (basale)
II: dopo anestetico e stimolazione nasale (riflessa)
III: dopo anestetico, stimolazione luce solare
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Workup Sr. Sjögren
TEST LACRIMALI
Turn-over lacrimale
Test diluizione (fluorescina e rosa bengala)
Titolazione lisozima e lattoferrina
Felcizzazione
Citologia ad impressione
Biomicroscopiainterferenziale
Osmolarità
Meniscometria
Meibomiometria
Spettrometria di massa
Citometria di flusso
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Terapia
Lacrime artificiali senza conservanti
Riduzione della componente acquosa
• Lacrime ad azione diluente:
Lacrime ipotoniche ogni 15 min, scarsa durata d’azione
Piccole confezioni di soluzione fisiologica
Occhiali specifici!
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Terapia
Lacrime artificiali senza conservanti
• Lacrime ad azione di volume:
Agenti addensanti che aumentano la viscosità e permanenza
Carbossimetilcellulosa, idrossietilcellulosa, polisaccaridi
(destrano, condroitinsolfato e ialuronato sodico)
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Terapia
Lacrime artificiali senza conservanti
• Lacrime ad azione stabilizzante:
Mucomimetici: rendere idrofila la superficie oculare, abbassare la tensione superficiale del liquido lacrimale e determinare densità ottimale per la permanenza
Alcol polivinilico, polivinilpirrolidone, polisaccaridi, esteri della cellulosa, carbocol
Resine gel trasparenti vischiosi che aderiscono alla cornea intrappolando acqua
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Terapia
• Siero autologo
• Trattamento antiinfiammatorio:
– FANS topici
– Ciclosporina A topica (0.05-1%)
– Corticosteroidi topici
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Terapia
• Integratori
(acidi grassi essenziali omega 3 e 6)
• Pilocarpina cloridrato sistemica/civimelina
• αInterferone
• Terapia ormonale-androgeni
• Tetraciclina
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Terapia
•
Punctal Plug
• LAC
• AMT
• Ricoprimento congiuntivale
• Tossina botulinica inducente ptosi
• Tarsorrafia