Orbital/ocular inflammatory involvement in VEXAS syndrome: Data from the international AIDA network VEXAS registry
VEXAS syndrome is a recently described monogenic autoinflammatory disease capable of manifesting itself with a wide array of organs and tissues involvement. Orbital/ocular inflammatory manifestations are frequently described in VEXAS patients. The objective of this study is to further describe orbit...
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| Veröffentlicht in: | Seminars in arthritis and rheumatism 2024-06, Vol.66, p.152430-152430, Article 152430 |
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| creator | Vitale, Antonio Caggiano, Valeria Martin-Nares, Eduardo Frassi, Micol Dagna, Lorenzo Hissaria, Pravin Sfriso, Paolo Hernández-Rodríguez, José Ruiz-Irastorza, Guillermo Monti, Sara Tufan, Abdurrahman Piga, Matteo Giardini, Henrique A Mayrink Lopalco, Giuseppe Viapiana, Ombretta De Paulis, Amato Triggianese, Paola Vitetta, Rosetta de-la-Torre, Alejandra Fonollosa, Alex Caroni, Federico Sota, Jurgen Conticini, Edoardo Sbalchiero, Jessica Renieri, Alessandra Casamassima, Giulia Wiesik-Szewczyk, Ewa Yildirim, Derya Hinojosa-Azaola, Andrea Crisafulli, Francesca Franceschini, Franco Campochiaro, Corrado Tomelleri, Alessandro Callisto, Alicia Beecher, Mark Bindoli, Sara Baggio, Chiara Gómez-Caverzaschi, Verónica Pelegrín, Laura Soto-Peleteiro, Adriana Milanesi, Alessandra Vasi, Ibrahim Cauli, Alberto Antonelli, Isabele Parente de Brito Iannone, Florenzo Bixio, Riccardo Casa, Francesca Della Mormile, Ilaria Gurnari, Carmelo Fiorenza, Alessia Mejia-Salgado, Germán Kawakami-Campos, Perla Ayumi Ragab, Gaafar Ciccia, Francesco Ruscitti, Piero Bocchia, Monica Balistreri, Alberto Tosi, Gian Marco Frediani, Bruno Cantarini, Luca Fabiani, Claudia |
| description | VEXAS syndrome is a recently described monogenic autoinflammatory disease capable of manifesting itself with a wide array of organs and tissues involvement. Orbital/ocular inflammatory manifestations are frequently described in VEXAS patients. The objective of this study is to further describe orbital/ocular conditions in VEXAS syndrome while investigating potential associations with other disease manifestations. In the present study, twenty-seven out of 59 (45.8 %) VEXAS patients showed an inflammatory orbital/ocular involvement during their clinical history. The most frequent orbital/ocular affections were represented by periorbital edema in 8 (13.6 %) cases, episcleritis in 5 (8.5 %) patients, scleritis in 5 (8.5 %) cases, uveitis in 4 (6.8 %) cases, conjunctivitis in 4 (6.8 %) cases, blepharitis in 3 (5.1 %) cases, orbital myositis in 2 (3.4 %) cases. A diagnosis of systemic immune-mediated disease was observed in 15 (55.6 %) cases, with relapsing polychondritis diagnosed in 12 patients. A significant association was observed between relapsing polychondritis and orbital/ocular involvement in VEXAS syndrome (Relative Risk: 2.37, 95 % C.I. 1.03–5.46, p = 0.048). Six deaths were observed in the whole cohort of patients after a median disease duration of 1.2 (IQR=5.35) years, 5 (83.3 %) of which showed orbital/ocular inflammatory involvement. In conclusion, this study confirms that orbital/ocular inflammatory involvement is a common finding in VEXAS patients, especially when relapsing polychondritis is diagnosed. This makes ophthalmologists a key figure in the diagnostic process of VEXAS syndrome. The high frequency of deaths observed in this study seems to suggest that patients with orbital/ocular involvement may require increased attention and more careful follow-up. |
| doi_str_mv | 10.1016/j.semarthrit.2024.152430 |
| format | Article |
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Orbital/ocular inflammatory manifestations are frequently described in VEXAS patients. The objective of this study is to further describe orbital/ocular conditions in VEXAS syndrome while investigating potential associations with other disease manifestations. In the present study, twenty-seven out of 59 (45.8 %) VEXAS patients showed an inflammatory orbital/ocular involvement during their clinical history. The most frequent orbital/ocular affections were represented by periorbital edema in 8 (13.6 %) cases, episcleritis in 5 (8.5 %) patients, scleritis in 5 (8.5 %) cases, uveitis in 4 (6.8 %) cases, conjunctivitis in 4 (6.8 %) cases, blepharitis in 3 (5.1 %) cases, orbital myositis in 2 (3.4 %) cases. A diagnosis of systemic immune-mediated disease was observed in 15 (55.6 %) cases, with relapsing polychondritis diagnosed in 12 patients. A significant association was observed between relapsing polychondritis and orbital/ocular involvement in VEXAS syndrome (Relative Risk: 2.37, 95 % C.I. 1.03–5.46, p = 0.048). Six deaths were observed in the whole cohort of patients after a median disease duration of 1.2 (IQR=5.35) years, 5 (83.3 %) of which showed orbital/ocular inflammatory involvement. In conclusion, this study confirms that orbital/ocular inflammatory involvement is a common finding in VEXAS patients, especially when relapsing polychondritis is diagnosed. This makes ophthalmologists a key figure in the diagnostic process of VEXAS syndrome. The high frequency of deaths observed in this study seems to suggest that patients with orbital/ocular involvement may require increased attention and more careful follow-up.</description><identifier>ISSN: 0049-0172</identifier><identifier>EISSN: 1532-866X</identifier><identifier>DOI: 10.1016/j.semarthrit.2024.152430</identifier><identifier>PMID: 38554594</identifier><language>eng</language><publisher>United States: Elsevier Inc</publisher><subject>Adolescent ; Adult ; Aged ; AIDA network VEXAS registry ; Autoinflammatory diseases ; Child ; Eye Diseases - epidemiology ; Female ; Hereditary Autoinflammatory Diseases - diagnosis ; Humans ; Male ; Middle Aged ; Myelodysplastic syndrome ; Orbital Diseases ; Polychondritis, Relapsing - complications ; Polychondritis, Relapsing - diagnosis ; Polychondritis, Relapsing - epidemiology ; Registries ; Relapsing polychondritis ; Scleritis ; Scleritis - diagnosis ; Scleritis - epidemiology ; Uveitis ; Young Adult</subject><ispartof>Seminars in arthritis and rheumatism, 2024-06, Vol.66, p.152430-152430, Article 152430</ispartof><rights>2024</rights><rights>Copyright © 2024. Published by Elsevier Inc.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c369t-c25cb46950fcc3fb8febdbb35ddf29b69ac32d5bdab709cbf03f647f6045903e3</cites><orcidid>0000-0002-7352-1275</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://www.sciencedirect.com/science/article/pii/S0049017224000702$$EHTML$$P50$$Gelsevier$$Hfree_for_read</linktohtml><link.rule.ids>314,776,780,3537,27901,27902,65534</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/38554594$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Vitale, Antonio</creatorcontrib><creatorcontrib>Caggiano, Valeria</creatorcontrib><creatorcontrib>Martin-Nares, Eduardo</creatorcontrib><creatorcontrib>Frassi, Micol</creatorcontrib><creatorcontrib>Dagna, Lorenzo</creatorcontrib><creatorcontrib>Hissaria, Pravin</creatorcontrib><creatorcontrib>Sfriso, Paolo</creatorcontrib><creatorcontrib>Hernández-Rodríguez, José</creatorcontrib><creatorcontrib>Ruiz-Irastorza, Guillermo</creatorcontrib><creatorcontrib>Monti, Sara</creatorcontrib><creatorcontrib>Tufan, Abdurrahman</creatorcontrib><creatorcontrib>Piga, Matteo</creatorcontrib><creatorcontrib>Giardini, Henrique A Mayrink</creatorcontrib><creatorcontrib>Lopalco, Giuseppe</creatorcontrib><creatorcontrib>Viapiana, Ombretta</creatorcontrib><creatorcontrib>De Paulis, Amato</creatorcontrib><creatorcontrib>Triggianese, Paola</creatorcontrib><creatorcontrib>Vitetta, Rosetta</creatorcontrib><creatorcontrib>de-la-Torre, Alejandra</creatorcontrib><creatorcontrib>Fonollosa, Alex</creatorcontrib><creatorcontrib>Caroni, Federico</creatorcontrib><creatorcontrib>Sota, Jurgen</creatorcontrib><creatorcontrib>Conticini, Edoardo</creatorcontrib><creatorcontrib>Sbalchiero, Jessica</creatorcontrib><creatorcontrib>Renieri, Alessandra</creatorcontrib><creatorcontrib>Casamassima, Giulia</creatorcontrib><creatorcontrib>Wiesik-Szewczyk, Ewa</creatorcontrib><creatorcontrib>Yildirim, Derya</creatorcontrib><creatorcontrib>Hinojosa-Azaola, Andrea</creatorcontrib><creatorcontrib>Crisafulli, Francesca</creatorcontrib><creatorcontrib>Franceschini, Franco</creatorcontrib><creatorcontrib>Campochiaro, Corrado</creatorcontrib><creatorcontrib>Tomelleri, Alessandro</creatorcontrib><creatorcontrib>Callisto, Alicia</creatorcontrib><creatorcontrib>Beecher, Mark</creatorcontrib><creatorcontrib>Bindoli, Sara</creatorcontrib><creatorcontrib>Baggio, Chiara</creatorcontrib><creatorcontrib>Gómez-Caverzaschi, Verónica</creatorcontrib><creatorcontrib>Pelegrín, Laura</creatorcontrib><creatorcontrib>Soto-Peleteiro, Adriana</creatorcontrib><creatorcontrib>Milanesi, Alessandra</creatorcontrib><creatorcontrib>Vasi, Ibrahim</creatorcontrib><creatorcontrib>Cauli, Alberto</creatorcontrib><creatorcontrib>Antonelli, Isabele Parente de Brito</creatorcontrib><creatorcontrib>Iannone, Florenzo</creatorcontrib><creatorcontrib>Bixio, Riccardo</creatorcontrib><creatorcontrib>Casa, Francesca Della</creatorcontrib><creatorcontrib>Mormile, Ilaria</creatorcontrib><creatorcontrib>Gurnari, Carmelo</creatorcontrib><creatorcontrib>Fiorenza, Alessia</creatorcontrib><creatorcontrib>Mejia-Salgado, Germán</creatorcontrib><creatorcontrib>Kawakami-Campos, Perla Ayumi</creatorcontrib><creatorcontrib>Ragab, Gaafar</creatorcontrib><creatorcontrib>Ciccia, Francesco</creatorcontrib><creatorcontrib>Ruscitti, Piero</creatorcontrib><creatorcontrib>Bocchia, Monica</creatorcontrib><creatorcontrib>Balistreri, Alberto</creatorcontrib><creatorcontrib>Tosi, Gian Marco</creatorcontrib><creatorcontrib>Frediani, Bruno</creatorcontrib><creatorcontrib>Cantarini, Luca</creatorcontrib><creatorcontrib>Fabiani, Claudia</creatorcontrib><title>Orbital/ocular inflammatory involvement in VEXAS syndrome: Data from the international AIDA network VEXAS registry</title><title>Seminars in arthritis and rheumatism</title><addtitle>Semin Arthritis Rheum</addtitle><description>VEXAS syndrome is a recently described monogenic autoinflammatory disease capable of manifesting itself with a wide array of organs and tissues involvement. Orbital/ocular inflammatory manifestations are frequently described in VEXAS patients. The objective of this study is to further describe orbital/ocular conditions in VEXAS syndrome while investigating potential associations with other disease manifestations. In the present study, twenty-seven out of 59 (45.8 %) VEXAS patients showed an inflammatory orbital/ocular involvement during their clinical history. The most frequent orbital/ocular affections were represented by periorbital edema in 8 (13.6 %) cases, episcleritis in 5 (8.5 %) patients, scleritis in 5 (8.5 %) cases, uveitis in 4 (6.8 %) cases, conjunctivitis in 4 (6.8 %) cases, blepharitis in 3 (5.1 %) cases, orbital myositis in 2 (3.4 %) cases. A diagnosis of systemic immune-mediated disease was observed in 15 (55.6 %) cases, with relapsing polychondritis diagnosed in 12 patients. A significant association was observed between relapsing polychondritis and orbital/ocular involvement in VEXAS syndrome (Relative Risk: 2.37, 95 % C.I. 1.03–5.46, p = 0.048). Six deaths were observed in the whole cohort of patients after a median disease duration of 1.2 (IQR=5.35) years, 5 (83.3 %) of which showed orbital/ocular inflammatory involvement. In conclusion, this study confirms that orbital/ocular inflammatory involvement is a common finding in VEXAS patients, especially when relapsing polychondritis is diagnosed. This makes ophthalmologists a key figure in the diagnostic process of VEXAS syndrome. The high frequency of deaths observed in this study seems to suggest that patients with orbital/ocular involvement may require increased attention and more careful follow-up.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Aged</subject><subject>AIDA network VEXAS registry</subject><subject>Autoinflammatory diseases</subject><subject>Child</subject><subject>Eye Diseases - epidemiology</subject><subject>Female</subject><subject>Hereditary Autoinflammatory Diseases - diagnosis</subject><subject>Humans</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Myelodysplastic syndrome</subject><subject>Orbital Diseases</subject><subject>Polychondritis, Relapsing - complications</subject><subject>Polychondritis, Relapsing - diagnosis</subject><subject>Polychondritis, Relapsing - epidemiology</subject><subject>Registries</subject><subject>Relapsing polychondritis</subject><subject>Scleritis</subject><subject>Scleritis - diagnosis</subject><subject>Scleritis - epidemiology</subject><subject>Uveitis</subject><subject>Young Adult</subject><issn>0049-0172</issn><issn>1532-866X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2024</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkE1vEzEQhi1ERdPCX0A-ctnUH2vvLrfQD1qpUg8tqDfLH2PqsLsuthOUf4-rBDj2NDPS885oHoQwJUtKqDxbLzNMOpWnFMqSEdYuqWAtJ2_QggrOml7Kx7doQUg7NIR27Bid5LwmhFJJunfomPdCtGJoFyjdJROKHs-i3Yw64TD7UU-TLjHt6rCN4xYmmEvt8ffLx9U9zrvZpTjBZ3yhi8a-9rg8QQUKpFmXEGc94tXNxQrPUH7H9PMQTPAj5JJ279GR12OGD4d6ir5dXT6cXze3d19vzle3jeVyKI1lwppWDoJ4a7k3vQfjjOHCOc8GIwdtOXPCOG06MljjCfey7bwk9TPCgZ-iT_u9zyn-2kAuagrZwjjqGeImK04YE53sOalov0dtijkn8Oo5hSp4pyhRL8bVWv03rl6Mq73xGv14uLIxE7h_wb-KK_BlD0D9dRsgqWwDzBZcSGCLcjG8fuUPVCWZ0Q</recordid><startdate>202406</startdate><enddate>202406</enddate><creator>Vitale, Antonio</creator><creator>Caggiano, Valeria</creator><creator>Martin-Nares, Eduardo</creator><creator>Frassi, 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Gian Marco</creator><creator>Frediani, Bruno</creator><creator>Cantarini, Luca</creator><creator>Fabiani, Claudia</creator><general>Elsevier Inc</general><scope>6I.</scope><scope>AAFTH</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0002-7352-1275</orcidid></search><sort><creationdate>202406</creationdate><title>Orbital/ocular inflammatory involvement in VEXAS syndrome: Data from the international AIDA network VEXAS registry</title><author>Vitale, Antonio ; Caggiano, Valeria ; Martin-Nares, Eduardo ; Frassi, Micol ; Dagna, Lorenzo ; Hissaria, Pravin ; Sfriso, Paolo ; Hernández-Rodríguez, José ; Ruiz-Irastorza, Guillermo ; Monti, Sara ; Tufan, Abdurrahman ; Piga, Matteo ; Giardini, Henrique A Mayrink ; Lopalco, Giuseppe ; Viapiana, Ombretta ; De Paulis, Amato ; Triggianese, Paola ; Vitetta, Rosetta ; de-la-Torre, Alejandra ; Fonollosa, Alex ; Caroni, Federico ; Sota, Jurgen ; Conticini, Edoardo ; Sbalchiero, Jessica ; Renieri, Alessandra ; Casamassima, Giulia ; Wiesik-Szewczyk, Ewa ; Yildirim, Derya ; Hinojosa-Azaola, Andrea ; Crisafulli, Francesca ; Franceschini, Franco ; Campochiaro, Corrado ; Tomelleri, Alessandro ; Callisto, Alicia ; Beecher, Mark ; Bindoli, Sara ; Baggio, Chiara ; Gómez-Caverzaschi, Verónica ; Pelegrín, Laura ; Soto-Peleteiro, Adriana ; Milanesi, Alessandra ; Vasi, Ibrahim ; Cauli, Alberto ; Antonelli, Isabele Parente de Brito ; Iannone, Florenzo ; Bixio, Riccardo ; Casa, Francesca Della ; Mormile, Ilaria ; Gurnari, Carmelo ; Fiorenza, Alessia ; Mejia-Salgado, Germán ; Kawakami-Campos, Perla Ayumi ; Ragab, Gaafar ; Ciccia, Francesco ; Ruscitti, Piero ; Bocchia, Monica ; Balistreri, Alberto ; Tosi, Gian Marco ; Frediani, Bruno ; Cantarini, Luca ; Fabiani, Claudia</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c369t-c25cb46950fcc3fb8febdbb35ddf29b69ac32d5bdab709cbf03f647f6045903e3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2024</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Aged</topic><topic>AIDA network VEXAS registry</topic><topic>Autoinflammatory diseases</topic><topic>Child</topic><topic>Eye Diseases - epidemiology</topic><topic>Female</topic><topic>Hereditary Autoinflammatory Diseases - diagnosis</topic><topic>Humans</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Myelodysplastic syndrome</topic><topic>Orbital Diseases</topic><topic>Polychondritis, Relapsing - complications</topic><topic>Polychondritis, Relapsing - diagnosis</topic><topic>Polychondritis, Relapsing - epidemiology</topic><topic>Registries</topic><topic>Relapsing polychondritis</topic><topic>Scleritis</topic><topic>Scleritis - diagnosis</topic><topic>Scleritis - epidemiology</topic><topic>Uveitis</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Vitale, Antonio</creatorcontrib><creatorcontrib>Caggiano, Valeria</creatorcontrib><creatorcontrib>Martin-Nares, Eduardo</creatorcontrib><creatorcontrib>Frassi, Micol</creatorcontrib><creatorcontrib>Dagna, Lorenzo</creatorcontrib><creatorcontrib>Hissaria, Pravin</creatorcontrib><creatorcontrib>Sfriso, Paolo</creatorcontrib><creatorcontrib>Hernández-Rodríguez, José</creatorcontrib><creatorcontrib>Ruiz-Irastorza, Guillermo</creatorcontrib><creatorcontrib>Monti, Sara</creatorcontrib><creatorcontrib>Tufan, Abdurrahman</creatorcontrib><creatorcontrib>Piga, Matteo</creatorcontrib><creatorcontrib>Giardini, Henrique A Mayrink</creatorcontrib><creatorcontrib>Lopalco, Giuseppe</creatorcontrib><creatorcontrib>Viapiana, Ombretta</creatorcontrib><creatorcontrib>De Paulis, Amato</creatorcontrib><creatorcontrib>Triggianese, Paola</creatorcontrib><creatorcontrib>Vitetta, Rosetta</creatorcontrib><creatorcontrib>de-la-Torre, Alejandra</creatorcontrib><creatorcontrib>Fonollosa, Alex</creatorcontrib><creatorcontrib>Caroni, Federico</creatorcontrib><creatorcontrib>Sota, Jurgen</creatorcontrib><creatorcontrib>Conticini, Edoardo</creatorcontrib><creatorcontrib>Sbalchiero, Jessica</creatorcontrib><creatorcontrib>Renieri, Alessandra</creatorcontrib><creatorcontrib>Casamassima, Giulia</creatorcontrib><creatorcontrib>Wiesik-Szewczyk, Ewa</creatorcontrib><creatorcontrib>Yildirim, Derya</creatorcontrib><creatorcontrib>Hinojosa-Azaola, Andrea</creatorcontrib><creatorcontrib>Crisafulli, Francesca</creatorcontrib><creatorcontrib>Franceschini, Franco</creatorcontrib><creatorcontrib>Campochiaro, Corrado</creatorcontrib><creatorcontrib>Tomelleri, Alessandro</creatorcontrib><creatorcontrib>Callisto, Alicia</creatorcontrib><creatorcontrib>Beecher, Mark</creatorcontrib><creatorcontrib>Bindoli, Sara</creatorcontrib><creatorcontrib>Baggio, Chiara</creatorcontrib><creatorcontrib>Gómez-Caverzaschi, Verónica</creatorcontrib><creatorcontrib>Pelegrín, Laura</creatorcontrib><creatorcontrib>Soto-Peleteiro, Adriana</creatorcontrib><creatorcontrib>Milanesi, Alessandra</creatorcontrib><creatorcontrib>Vasi, Ibrahim</creatorcontrib><creatorcontrib>Cauli, Alberto</creatorcontrib><creatorcontrib>Antonelli, Isabele Parente de Brito</creatorcontrib><creatorcontrib>Iannone, Florenzo</creatorcontrib><creatorcontrib>Bixio, Riccardo</creatorcontrib><creatorcontrib>Casa, Francesca Della</creatorcontrib><creatorcontrib>Mormile, Ilaria</creatorcontrib><creatorcontrib>Gurnari, Carmelo</creatorcontrib><creatorcontrib>Fiorenza, Alessia</creatorcontrib><creatorcontrib>Mejia-Salgado, Germán</creatorcontrib><creatorcontrib>Kawakami-Campos, Perla Ayumi</creatorcontrib><creatorcontrib>Ragab, Gaafar</creatorcontrib><creatorcontrib>Ciccia, Francesco</creatorcontrib><creatorcontrib>Ruscitti, Piero</creatorcontrib><creatorcontrib>Bocchia, Monica</creatorcontrib><creatorcontrib>Balistreri, Alberto</creatorcontrib><creatorcontrib>Tosi, Gian Marco</creatorcontrib><creatorcontrib>Frediani, Bruno</creatorcontrib><creatorcontrib>Cantarini, Luca</creatorcontrib><creatorcontrib>Fabiani, Claudia</creatorcontrib><collection>ScienceDirect Open Access Titles</collection><collection>Elsevier:ScienceDirect:Open Access</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Seminars in arthritis and rheumatism</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Vitale, Antonio</au><au>Caggiano, Valeria</au><au>Martin-Nares, Eduardo</au><au>Frassi, Micol</au><au>Dagna, Lorenzo</au><au>Hissaria, Pravin</au><au>Sfriso, Paolo</au><au>Hernández-Rodríguez, José</au><au>Ruiz-Irastorza, Guillermo</au><au>Monti, Sara</au><au>Tufan, Abdurrahman</au><au>Piga, Matteo</au><au>Giardini, Henrique A Mayrink</au><au>Lopalco, Giuseppe</au><au>Viapiana, Ombretta</au><au>De Paulis, Amato</au><au>Triggianese, Paola</au><au>Vitetta, Rosetta</au><au>de-la-Torre, Alejandra</au><au>Fonollosa, Alex</au><au>Caroni, Federico</au><au>Sota, Jurgen</au><au>Conticini, Edoardo</au><au>Sbalchiero, Jessica</au><au>Renieri, Alessandra</au><au>Casamassima, Giulia</au><au>Wiesik-Szewczyk, Ewa</au><au>Yildirim, Derya</au><au>Hinojosa-Azaola, Andrea</au><au>Crisafulli, Francesca</au><au>Franceschini, Franco</au><au>Campochiaro, Corrado</au><au>Tomelleri, Alessandro</au><au>Callisto, Alicia</au><au>Beecher, Mark</au><au>Bindoli, Sara</au><au>Baggio, Chiara</au><au>Gómez-Caverzaschi, Verónica</au><au>Pelegrín, Laura</au><au>Soto-Peleteiro, Adriana</au><au>Milanesi, Alessandra</au><au>Vasi, Ibrahim</au><au>Cauli, Alberto</au><au>Antonelli, Isabele Parente de Brito</au><au>Iannone, Florenzo</au><au>Bixio, Riccardo</au><au>Casa, Francesca Della</au><au>Mormile, Ilaria</au><au>Gurnari, Carmelo</au><au>Fiorenza, Alessia</au><au>Mejia-Salgado, Germán</au><au>Kawakami-Campos, Perla Ayumi</au><au>Ragab, Gaafar</au><au>Ciccia, Francesco</au><au>Ruscitti, Piero</au><au>Bocchia, Monica</au><au>Balistreri, Alberto</au><au>Tosi, Gian Marco</au><au>Frediani, Bruno</au><au>Cantarini, Luca</au><au>Fabiani, Claudia</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Orbital/ocular inflammatory involvement in VEXAS syndrome: Data from the international AIDA network VEXAS registry</atitle><jtitle>Seminars in arthritis and rheumatism</jtitle><addtitle>Semin Arthritis Rheum</addtitle><date>2024-06</date><risdate>2024</risdate><volume>66</volume><spage>152430</spage><epage>152430</epage><pages>152430-152430</pages><artnum>152430</artnum><issn>0049-0172</issn><eissn>1532-866X</eissn><abstract>VEXAS syndrome is a recently described monogenic autoinflammatory disease capable of manifesting itself with a wide array of organs and tissues involvement. Orbital/ocular inflammatory manifestations are frequently described in VEXAS patients. The objective of this study is to further describe orbital/ocular conditions in VEXAS syndrome while investigating potential associations with other disease manifestations. In the present study, twenty-seven out of 59 (45.8 %) VEXAS patients showed an inflammatory orbital/ocular involvement during their clinical history. The most frequent orbital/ocular affections were represented by periorbital edema in 8 (13.6 %) cases, episcleritis in 5 (8.5 %) patients, scleritis in 5 (8.5 %) cases, uveitis in 4 (6.8 %) cases, conjunctivitis in 4 (6.8 %) cases, blepharitis in 3 (5.1 %) cases, orbital myositis in 2 (3.4 %) cases. A diagnosis of systemic immune-mediated disease was observed in 15 (55.6 %) cases, with relapsing polychondritis diagnosed in 12 patients. A significant association was observed between relapsing polychondritis and orbital/ocular involvement in VEXAS syndrome (Relative Risk: 2.37, 95 % C.I. 1.03–5.46, p = 0.048). Six deaths were observed in the whole cohort of patients after a median disease duration of 1.2 (IQR=5.35) years, 5 (83.3 %) of which showed orbital/ocular inflammatory involvement. In conclusion, this study confirms that orbital/ocular inflammatory involvement is a common finding in VEXAS patients, especially when relapsing polychondritis is diagnosed. This makes ophthalmologists a key figure in the diagnostic process of VEXAS syndrome. The high frequency of deaths observed in this study seems to suggest that patients with orbital/ocular involvement may require increased attention and more careful follow-up.</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>38554594</pmid><doi>10.1016/j.semarthrit.2024.152430</doi><tpages>1</tpages><orcidid>https://orcid.org/0000-0002-7352-1275</orcidid><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0049-0172 |
| ispartof | Seminars in arthritis and rheumatism, 2024-06, Vol.66, p.152430-152430, Article 152430 |
| issn | 0049-0172 1532-866X |
| language | eng |
| recordid | cdi_proquest_miscellaneous_3022576830 |
| source | MEDLINE; Elsevier ScienceDirect Journals Complete |
| subjects | Adolescent Adult Aged AIDA network VEXAS registry Autoinflammatory diseases Child Eye Diseases - epidemiology Female Hereditary Autoinflammatory Diseases - diagnosis Humans Male Middle Aged Myelodysplastic syndrome Orbital Diseases Polychondritis, Relapsing - complications Polychondritis, Relapsing - diagnosis Polychondritis, Relapsing - epidemiology Registries Relapsing polychondritis Scleritis Scleritis - diagnosis Scleritis - epidemiology Uveitis Young Adult |
| title | Orbital/ocular inflammatory involvement in VEXAS syndrome: Data from the international AIDA network VEXAS registry |
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