Ormond’s Disease – 26 Years of Experience
at One Centre
Università Campus Bio-Medico di RomaLavoro di Emilio Pagano e Giuseppe di Nuzzo
UNA RICERCA DI 26 ANNI
studio sulla malattia di Ormond condotto dall'ospedale Na Homolce di Praga (CZ)
- Cos'è la malattia di Ormond
- Eziologia
- Diagnosi
- Trattamento clinico- farmaceutico
- Terapie ed effetti
Cos'è la malattia di Ormond?
(anche detta fibrosi retroperitoneale ideopatica o IRF)
33,7% presenta IgG4-RD
Infiammazione retroperitonealeEziologia poco chiara
1,3 ogni 100.000 persone
>30 IgG4+ per campo ad alto ingrandimento
Incidenzamaschio: femmina= 2:1
IgG4+/IgG+ >40%
Eziologia sconosciuta ma...
- Irregolarità th2 e tr-reg
- aumento rilascio citochine (interleuchine)
- Processo infiammatorio e probabili ostruzioni dovute a IgG4 e TGF- β
- Infiammazione RP, aneurisma di arterie iliache e aorta addominale, insufficienza renale
Diagnosi
- Analisi biochimica, immunologica e test microbiologici
- metodi di imaging (ultrasuoni, PET/TC
- Screening laboratorio e pannello biochimico
- Screening per epatite B e C
- Livelli IgG (IgG1-IgG4)
- Sedimentazione eritrociti (VES)
135 "pazienti" iniziali
5 avevano un tumore retroperitoneale
5 non presentavano attività di malattia
34 non presentavano malattia di Ormond
8 avevano aneurisma aortico infiammatorio addominale
82 pazienti con malattia di Ormond
25 con malattie autoimmuni associate
22 con Anticorpi antinucleo
53 pazienti con ostruzione uretrale
28 con livelli elevati di IgG4
Trattamenti utilizzati
Chirurgico
- Stent uretrale
- Nefrostomia
- Stent delle arterie
Terapia immunosoppressiva
- Corticosteroidi (Azatioprina)
- Micofenolato Mofetile
- Rituximab
AZIONE DEI CORTICOSTEROIDI
Trans repressione
Cambio conformazione
Inibizione fattori chiave dell'infiammazione come NF-kb e AP-1 (IL-1, IL-2, IL-6)
Si legano a GR, la proteina chaperon come conseguenza rompe il suo legame con GR
RITUXIMAB
Il Rituximab non entra nella cellula, ma innesca vie di segnalazione intracellulare attraverso il legame con il CD20 (una proteina transmembrana idrofobica sui linfociti B).
Legame con CD20
Marca i linfociti B
Citotossicità cellula mediata
Riduzione linfociti B(Apoptosi)
Attivazione complesso
GRAZIE PER L'ATTENZIONE!
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Ormond’s Disease – 26 Years of Experience at One Centre
Emilio Pagano
Created on April 21, 2026
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Transcript
Ormond’s Disease – 26 Years of Experience at One Centre
Università Campus Bio-Medico di RomaLavoro di Emilio Pagano e Giuseppe di Nuzzo
UNA RICERCA DI 26 ANNI
studio sulla malattia di Ormond condotto dall'ospedale Na Homolce di Praga (CZ)
Cos'è la malattia di Ormond?
(anche detta fibrosi retroperitoneale ideopatica o IRF)
33,7% presenta IgG4-RD
Infiammazione retroperitonealeEziologia poco chiara
1,3 ogni 100.000 persone
>30 IgG4+ per campo ad alto ingrandimento
Incidenzamaschio: femmina= 2:1
IgG4+/IgG+ >40%
Eziologia sconosciuta ma...
Diagnosi
135 "pazienti" iniziali
5 avevano un tumore retroperitoneale
5 non presentavano attività di malattia
34 non presentavano malattia di Ormond
8 avevano aneurisma aortico infiammatorio addominale
82 pazienti con malattia di Ormond
25 con malattie autoimmuni associate
22 con Anticorpi antinucleo
53 pazienti con ostruzione uretrale
28 con livelli elevati di IgG4
Trattamenti utilizzati
Chirurgico
Terapia immunosoppressiva
AZIONE DEI CORTICOSTEROIDI
Trans repressione
Cambio conformazione
Inibizione fattori chiave dell'infiammazione come NF-kb e AP-1 (IL-1, IL-2, IL-6)
Si legano a GR, la proteina chaperon come conseguenza rompe il suo legame con GR
RITUXIMAB
Il Rituximab non entra nella cellula, ma innesca vie di segnalazione intracellulare attraverso il legame con il CD20 (una proteina transmembrana idrofobica sui linfociti B).
Legame con CD20
Marca i linfociti B
Citotossicità cellula mediata
Riduzione linfociti B(Apoptosi)
Attivazione complesso
GRAZIE PER L'ATTENZIONE!
Write a title here
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GO
Lorem ipsum dolor sit amet, consectetur adipiscing elit, sed do eiusmod tempor incididunt.
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Lorem ipsum dolor sit amet, consectetur
Lorem ipsum dolor sit amet, consectetur adipiscing elit.
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Lorem ipsum dolor sit amet, consectetur adipiscing elit
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Lorem ipsum dolor sit amet, consectetur
Lorem ipsum dolor sit amet, consectetur adipiscing elit, sed do eiusmod tempor incididunt.
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Lorem ipsum dolor sit amet, consectetur
Lorem Ipsum
Lorem Ipsum
Lorem ipsum dolor sit
Lorem ipsum dolor sit
Lorem Ipsum
Lorem Ipsum
Lorem ipsum dolor sit
Lorem ipsum dolor sit
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