Budd–Chiari syndrome in Behçet’s disease: a retrospective multicenter study
Objective To compare the clinical features, laboratory findings, and prognosis of Behçet’s disease (BD) patients with and without Budd–Chiari syndrome (BCS). Methods This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD pat...
Ausführliche Beschreibung
Autor*in: |
Akyol, Lütfi [verfasserIn] |
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E-Artikel |
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Sprache: |
Englisch |
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2021 |
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Anmerkung: |
© International League of Associations for Rheumatology (ILAR) 2021 |
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Übergeordnetes Werk: |
Enthalten in: Clinical rheumatology - London : Springer, 1982, 41(2021), 1 vom: 09. Aug., Seite 177-186 |
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Übergeordnetes Werk: |
volume:41 ; year:2021 ; number:1 ; day:09 ; month:08 ; pages:177-186 |
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DOI / URN: |
10.1007/s10067-021-05878-2 |
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Katalog-ID: |
SPR045865671 |
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520 | |a Objective To compare the clinical features, laboratory findings, and prognosis of Behçet’s disease (BD) patients with and without Budd–Chiari syndrome (BCS). Methods This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017. Results Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.005 and p = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p < 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14–47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%). Conclusion To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. Key Points•Mortality rate is higher in BD-associated BCS patients with IVC involvement.•Chronic and silent form of BD-associated BCS has a better prognosis.•The main treatment options are corticosteroids and immunosuppressive agents, whereas anticoagulant treatment remains controversial. | ||
650 | 4 | |a Behçet’s disease |7 (dpeaa)DE-He213 | |
650 | 4 | |a Budd–Chiari syndrome |7 (dpeaa)DE-He213 | |
650 | 4 | |a Inferior vena cava |7 (dpeaa)DE-He213 | |
650 | 4 | |a Prognosis |7 (dpeaa)DE-He213 | |
650 | 4 | |a Thrombosis |7 (dpeaa)DE-He213 | |
700 | 1 | |a Toz, Bahtiyar |4 aut | |
700 | 1 | |a Bayındır, Özün |4 aut | |
700 | 1 | |a Zengin, Orhan |4 aut | |
700 | 1 | |a Cansu, DöndüÜsküdar |4 aut | |
700 | 1 | |a Yiğit, Murat |4 aut | |
700 | 1 | |a Çetin, Gözde Yıldırım |4 aut | |
700 | 1 | |a Omma, Ahmet |4 aut | |
700 | 1 | |a Erden, Abdulsamet |4 aut | |
700 | 1 | |a Küçükşahin, Orhan |4 aut | |
700 | 1 | |a Altuner, Mehmet Şakir |4 aut | |
700 | 1 | |a Çorba, BurçinŞeyda |4 aut | |
700 | 1 | |a Ünal, Ali Uğur |4 aut | |
700 | 1 | |a Küçük, Hamit |4 aut | |
700 | 1 | |a Küçük, Adem |4 aut | |
700 | 1 | |a Balkarli, Ayşe |4 aut | |
700 | 1 | |a Gönüllü, Emel |4 aut | |
700 | 1 | |a Tufan, Ayşe Nur |4 aut | |
700 | 1 | |a Bakırcı, Sibel |4 aut | |
700 | 1 | |a Öner, Sibel Yılmaz |4 aut | |
700 | 1 | |a Balcı, Mehmet Ali |4 aut | |
700 | 1 | |a Kobak, Şenol |4 aut | |
700 | 1 | |a Yazıcı, Ayten |4 aut | |
700 | 1 | |a Özgen, Metin |4 aut | |
700 | 1 | |a Şahin, Ali |4 aut | |
700 | 1 | |a Koca, Süleyman Serdar |4 aut | |
700 | 1 | |a Erer, Burak |4 aut | |
700 | 1 | |a Gül, Ahmet |4 aut | |
700 | 1 | |a Aksu, Kenan |4 aut | |
700 | 1 | |a Keser, Gökhan |4 aut | |
700 | 1 | |a Onat, Ahmet Mesut |4 aut | |
700 | 1 | |a Kısacık, Bünyamin |4 aut | |
700 | 1 | |a Kaşifoğlu, Timuçin |4 aut | |
700 | 1 | |a Çefle, Ayşe |4 aut | |
700 | 1 | |a Kalyoncu, Umut |4 aut | |
700 | 1 | |a Sayarlıoğlu, Mehmet |4 aut | |
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10.1007/s10067-021-05878-2 doi (DE-627)SPR045865671 (SPR)s10067-021-05878-2-e DE-627 ger DE-627 rakwb eng Akyol, Lütfi verfasserin aut Budd–Chiari syndrome in Behçet’s disease: a retrospective multicenter study 2021 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © International League of Associations for Rheumatology (ILAR) 2021 Objective To compare the clinical features, laboratory findings, and prognosis of Behçet’s disease (BD) patients with and without Budd–Chiari syndrome (BCS). Methods This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017. Results Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.005 and p = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p < 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14–47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%). Conclusion To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. Key Points•Mortality rate is higher in BD-associated BCS patients with IVC involvement.•Chronic and silent form of BD-associated BCS has a better prognosis.•The main treatment options are corticosteroids and immunosuppressive agents, whereas anticoagulant treatment remains controversial. Behçet’s disease (dpeaa)DE-He213 Budd–Chiari syndrome (dpeaa)DE-He213 Inferior vena cava (dpeaa)DE-He213 Prognosis (dpeaa)DE-He213 Thrombosis (dpeaa)DE-He213 Toz, Bahtiyar aut Bayındır, Özün aut Zengin, Orhan aut Cansu, DöndüÜsküdar aut Yiğit, Murat aut Çetin, Gözde Yıldırım aut Omma, Ahmet aut Erden, Abdulsamet aut Küçükşahin, Orhan aut Altuner, Mehmet Şakir aut Çorba, BurçinŞeyda aut Ünal, Ali Uğur aut Küçük, Hamit aut Küçük, Adem aut Balkarli, Ayşe aut Gönüllü, Emel aut Tufan, Ayşe Nur aut Bakırcı, Sibel aut Öner, Sibel Yılmaz aut Balcı, Mehmet Ali aut Kobak, Şenol aut Yazıcı, Ayten aut Özgen, Metin aut Şahin, Ali aut Koca, Süleyman Serdar aut Erer, Burak aut Gül, Ahmet aut Aksu, Kenan aut Keser, Gökhan aut Onat, Ahmet Mesut aut Kısacık, Bünyamin aut Kaşifoğlu, Timuçin aut Çefle, Ayşe aut Kalyoncu, Umut aut Sayarlıoğlu, Mehmet aut Enthalten in Clinical rheumatology London : Springer, 1982 41(2021), 1 vom: 09. Aug., Seite 177-186 (DE-627)27159909X (DE-600)1480901-1 1434-9949 nnns volume:41 year:2021 number:1 day:09 month:08 pages:177-186 https://dx.doi.org/10.1007/s10067-021-05878-2 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 41 2021 1 09 08 177-186 |
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10.1007/s10067-021-05878-2 doi (DE-627)SPR045865671 (SPR)s10067-021-05878-2-e DE-627 ger DE-627 rakwb eng Akyol, Lütfi verfasserin aut Budd–Chiari syndrome in Behçet’s disease: a retrospective multicenter study 2021 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © International League of Associations for Rheumatology (ILAR) 2021 Objective To compare the clinical features, laboratory findings, and prognosis of Behçet’s disease (BD) patients with and without Budd–Chiari syndrome (BCS). Methods This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017. Results Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.005 and p = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p < 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14–47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%). Conclusion To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. Key Points•Mortality rate is higher in BD-associated BCS patients with IVC involvement.•Chronic and silent form of BD-associated BCS has a better prognosis.•The main treatment options are corticosteroids and immunosuppressive agents, whereas anticoagulant treatment remains controversial. Behçet’s disease (dpeaa)DE-He213 Budd–Chiari syndrome (dpeaa)DE-He213 Inferior vena cava (dpeaa)DE-He213 Prognosis (dpeaa)DE-He213 Thrombosis (dpeaa)DE-He213 Toz, Bahtiyar aut Bayındır, Özün aut Zengin, Orhan aut Cansu, DöndüÜsküdar aut Yiğit, Murat aut Çetin, Gözde Yıldırım aut Omma, Ahmet aut Erden, Abdulsamet aut Küçükşahin, Orhan aut Altuner, Mehmet Şakir aut Çorba, BurçinŞeyda aut Ünal, Ali Uğur aut Küçük, Hamit aut Küçük, Adem aut Balkarli, Ayşe aut Gönüllü, Emel aut Tufan, Ayşe Nur aut Bakırcı, Sibel aut Öner, Sibel Yılmaz aut Balcı, Mehmet Ali aut Kobak, Şenol aut Yazıcı, Ayten aut Özgen, Metin aut Şahin, Ali aut Koca, Süleyman Serdar aut Erer, Burak aut Gül, Ahmet aut Aksu, Kenan aut Keser, Gökhan aut Onat, Ahmet Mesut aut Kısacık, Bünyamin aut Kaşifoğlu, Timuçin aut Çefle, Ayşe aut Kalyoncu, Umut aut Sayarlıoğlu, Mehmet aut Enthalten in Clinical rheumatology London : Springer, 1982 41(2021), 1 vom: 09. Aug., Seite 177-186 (DE-627)27159909X (DE-600)1480901-1 1434-9949 nnns volume:41 year:2021 number:1 day:09 month:08 pages:177-186 https://dx.doi.org/10.1007/s10067-021-05878-2 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 41 2021 1 09 08 177-186 |
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10.1007/s10067-021-05878-2 doi (DE-627)SPR045865671 (SPR)s10067-021-05878-2-e DE-627 ger DE-627 rakwb eng Akyol, Lütfi verfasserin aut Budd–Chiari syndrome in Behçet’s disease: a retrospective multicenter study 2021 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © International League of Associations for Rheumatology (ILAR) 2021 Objective To compare the clinical features, laboratory findings, and prognosis of Behçet’s disease (BD) patients with and without Budd–Chiari syndrome (BCS). Methods This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017. Results Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.005 and p = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p < 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14–47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%). Conclusion To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. Key Points•Mortality rate is higher in BD-associated BCS patients with IVC involvement.•Chronic and silent form of BD-associated BCS has a better prognosis.•The main treatment options are corticosteroids and immunosuppressive agents, whereas anticoagulant treatment remains controversial. Behçet’s disease (dpeaa)DE-He213 Budd–Chiari syndrome (dpeaa)DE-He213 Inferior vena cava (dpeaa)DE-He213 Prognosis (dpeaa)DE-He213 Thrombosis (dpeaa)DE-He213 Toz, Bahtiyar aut Bayındır, Özün aut Zengin, Orhan aut Cansu, DöndüÜsküdar aut Yiğit, Murat aut Çetin, Gözde Yıldırım aut Omma, Ahmet aut Erden, Abdulsamet aut Küçükşahin, Orhan aut Altuner, Mehmet Şakir aut Çorba, BurçinŞeyda aut Ünal, Ali Uğur aut Küçük, Hamit aut Küçük, Adem aut Balkarli, Ayşe aut Gönüllü, Emel aut Tufan, Ayşe Nur aut Bakırcı, Sibel aut Öner, Sibel Yılmaz aut Balcı, Mehmet Ali aut Kobak, Şenol aut Yazıcı, Ayten aut Özgen, Metin aut Şahin, Ali aut Koca, Süleyman Serdar aut Erer, Burak aut Gül, Ahmet aut Aksu, Kenan aut Keser, Gökhan aut Onat, Ahmet Mesut aut Kısacık, Bünyamin aut Kaşifoğlu, Timuçin aut Çefle, Ayşe aut Kalyoncu, Umut aut Sayarlıoğlu, Mehmet aut Enthalten in Clinical rheumatology London : Springer, 1982 41(2021), 1 vom: 09. Aug., Seite 177-186 (DE-627)27159909X (DE-600)1480901-1 1434-9949 nnns volume:41 year:2021 number:1 day:09 month:08 pages:177-186 https://dx.doi.org/10.1007/s10067-021-05878-2 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 41 2021 1 09 08 177-186 |
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10.1007/s10067-021-05878-2 doi (DE-627)SPR045865671 (SPR)s10067-021-05878-2-e DE-627 ger DE-627 rakwb eng Akyol, Lütfi verfasserin aut Budd–Chiari syndrome in Behçet’s disease: a retrospective multicenter study 2021 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © International League of Associations for Rheumatology (ILAR) 2021 Objective To compare the clinical features, laboratory findings, and prognosis of Behçet’s disease (BD) patients with and without Budd–Chiari syndrome (BCS). Methods This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017. Results Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.005 and p = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p < 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14–47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%). Conclusion To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. Key Points•Mortality rate is higher in BD-associated BCS patients with IVC involvement.•Chronic and silent form of BD-associated BCS has a better prognosis.•The main treatment options are corticosteroids and immunosuppressive agents, whereas anticoagulant treatment remains controversial. Behçet’s disease (dpeaa)DE-He213 Budd–Chiari syndrome (dpeaa)DE-He213 Inferior vena cava (dpeaa)DE-He213 Prognosis (dpeaa)DE-He213 Thrombosis (dpeaa)DE-He213 Toz, Bahtiyar aut Bayındır, Özün aut Zengin, Orhan aut Cansu, DöndüÜsküdar aut Yiğit, Murat aut Çetin, Gözde Yıldırım aut Omma, Ahmet aut Erden, Abdulsamet aut Küçükşahin, Orhan aut Altuner, Mehmet Şakir aut Çorba, BurçinŞeyda aut Ünal, Ali Uğur aut Küçük, Hamit aut Küçük, Adem aut Balkarli, Ayşe aut Gönüllü, Emel aut Tufan, Ayşe Nur aut Bakırcı, Sibel aut Öner, Sibel Yılmaz aut Balcı, Mehmet Ali aut Kobak, Şenol aut Yazıcı, Ayten aut Özgen, Metin aut Şahin, Ali aut Koca, Süleyman Serdar aut Erer, Burak aut Gül, Ahmet aut Aksu, Kenan aut Keser, Gökhan aut Onat, Ahmet Mesut aut Kısacık, Bünyamin aut Kaşifoğlu, Timuçin aut Çefle, Ayşe aut Kalyoncu, Umut aut Sayarlıoğlu, Mehmet aut Enthalten in Clinical rheumatology London : Springer, 1982 41(2021), 1 vom: 09. Aug., Seite 177-186 (DE-627)27159909X (DE-600)1480901-1 1434-9949 nnns volume:41 year:2021 number:1 day:09 month:08 pages:177-186 https://dx.doi.org/10.1007/s10067-021-05878-2 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 41 2021 1 09 08 177-186 |
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10.1007/s10067-021-05878-2 doi (DE-627)SPR045865671 (SPR)s10067-021-05878-2-e DE-627 ger DE-627 rakwb eng Akyol, Lütfi verfasserin aut Budd–Chiari syndrome in Behçet’s disease: a retrospective multicenter study 2021 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © International League of Associations for Rheumatology (ILAR) 2021 Objective To compare the clinical features, laboratory findings, and prognosis of Behçet’s disease (BD) patients with and without Budd–Chiari syndrome (BCS). Methods This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017. Results Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.005 and p = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p < 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14–47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%). Conclusion To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. Key Points•Mortality rate is higher in BD-associated BCS patients with IVC involvement.•Chronic and silent form of BD-associated BCS has a better prognosis.•The main treatment options are corticosteroids and immunosuppressive agents, whereas anticoagulant treatment remains controversial. Behçet’s disease (dpeaa)DE-He213 Budd–Chiari syndrome (dpeaa)DE-He213 Inferior vena cava (dpeaa)DE-He213 Prognosis (dpeaa)DE-He213 Thrombosis (dpeaa)DE-He213 Toz, Bahtiyar aut Bayındır, Özün aut Zengin, Orhan aut Cansu, DöndüÜsküdar aut Yiğit, Murat aut Çetin, Gözde Yıldırım aut Omma, Ahmet aut Erden, Abdulsamet aut Küçükşahin, Orhan aut Altuner, Mehmet Şakir aut Çorba, BurçinŞeyda aut Ünal, Ali Uğur aut Küçük, Hamit aut Küçük, Adem aut Balkarli, Ayşe aut Gönüllü, Emel aut Tufan, Ayşe Nur aut Bakırcı, Sibel aut Öner, Sibel Yılmaz aut Balcı, Mehmet Ali aut Kobak, Şenol aut Yazıcı, Ayten aut Özgen, Metin aut Şahin, Ali aut Koca, Süleyman Serdar aut Erer, Burak aut Gül, Ahmet aut Aksu, Kenan aut Keser, Gökhan aut Onat, Ahmet Mesut aut Kısacık, Bünyamin aut Kaşifoğlu, Timuçin aut Çefle, Ayşe aut Kalyoncu, Umut aut Sayarlıoğlu, Mehmet aut Enthalten in Clinical rheumatology London : Springer, 1982 41(2021), 1 vom: 09. Aug., Seite 177-186 (DE-627)27159909X (DE-600)1480901-1 1434-9949 nnns volume:41 year:2021 number:1 day:09 month:08 pages:177-186 https://dx.doi.org/10.1007/s10067-021-05878-2 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 41 2021 1 09 08 177-186 |
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Enthalten in Clinical rheumatology 41(2021), 1 vom: 09. Aug., Seite 177-186 volume:41 year:2021 number:1 day:09 month:08 pages:177-186 |
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Enthalten in Clinical rheumatology 41(2021), 1 vom: 09. Aug., Seite 177-186 volume:41 year:2021 number:1 day:09 month:08 pages:177-186 |
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Clinical rheumatology |
authorswithroles_txt_mv |
Akyol, Lütfi @@aut@@ Toz, Bahtiyar @@aut@@ Bayındır, Özün @@aut@@ Zengin, Orhan @@aut@@ Cansu, DöndüÜsküdar @@aut@@ Yiğit, Murat @@aut@@ Çetin, Gözde Yıldırım @@aut@@ Omma, Ahmet @@aut@@ Erden, Abdulsamet @@aut@@ Küçükşahin, Orhan @@aut@@ Altuner, Mehmet Şakir @@aut@@ Çorba, BurçinŞeyda @@aut@@ Ünal, Ali Uğur @@aut@@ Küçük, Hamit @@aut@@ Küçük, Adem @@aut@@ Balkarli, Ayşe @@aut@@ Gönüllü, Emel @@aut@@ Tufan, Ayşe Nur @@aut@@ Bakırcı, Sibel @@aut@@ Öner, Sibel Yılmaz @@aut@@ Balcı, Mehmet Ali @@aut@@ Kobak, Şenol @@aut@@ Yazıcı, Ayten @@aut@@ Özgen, Metin @@aut@@ Şahin, Ali @@aut@@ Koca, Süleyman Serdar @@aut@@ Erer, Burak @@aut@@ Gül, Ahmet @@aut@@ Aksu, Kenan @@aut@@ Keser, Gökhan @@aut@@ Onat, Ahmet Mesut @@aut@@ Kısacık, Bünyamin @@aut@@ Kaşifoğlu, Timuçin @@aut@@ Çefle, Ayşe @@aut@@ Kalyoncu, Umut @@aut@@ Sayarlıoğlu, Mehmet @@aut@@ |
publishDateDaySort_date |
2021-08-09T00:00:00Z |
hierarchy_top_id |
27159909X |
id |
SPR045865671 |
language_de |
englisch |
fullrecord |
<?xml version="1.0" encoding="UTF-8"?><collection xmlns="http://www.loc.gov/MARC21/slim"><record><leader>01000caa a22002652 4500</leader><controlfield tag="001">SPR045865671</controlfield><controlfield tag="003">DE-627</controlfield><controlfield tag="005">20230519104212.0</controlfield><controlfield tag="007">cr uuu---uuuuu</controlfield><controlfield tag="008">220104s2021 xx |||||o 00| ||eng c</controlfield><datafield tag="024" ind1="7" ind2=" "><subfield code="a">10.1007/s10067-021-05878-2</subfield><subfield code="2">doi</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(DE-627)SPR045865671</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(SPR)s10067-021-05878-2-e</subfield></datafield><datafield tag="040" ind1=" " ind2=" "><subfield code="a">DE-627</subfield><subfield code="b">ger</subfield><subfield code="c">DE-627</subfield><subfield code="e">rakwb</subfield></datafield><datafield tag="041" ind1=" " ind2=" "><subfield code="a">eng</subfield></datafield><datafield tag="100" ind1="1" ind2=" "><subfield code="a">Akyol, Lütfi</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="245" ind1="1" ind2="0"><subfield code="a">Budd–Chiari syndrome in Behçet’s disease: a retrospective multicenter study</subfield></datafield><datafield tag="264" ind1=" " ind2="1"><subfield code="c">2021</subfield></datafield><datafield tag="336" ind1=" " ind2=" "><subfield code="a">Text</subfield><subfield code="b">txt</subfield><subfield code="2">rdacontent</subfield></datafield><datafield tag="337" ind1=" " ind2=" "><subfield code="a">Computermedien</subfield><subfield code="b">c</subfield><subfield code="2">rdamedia</subfield></datafield><datafield tag="338" ind1=" " ind2=" "><subfield code="a">Online-Ressource</subfield><subfield code="b">cr</subfield><subfield code="2">rdacarrier</subfield></datafield><datafield tag="500" ind1=" " ind2=" "><subfield code="a">© International League of Associations for Rheumatology (ILAR) 2021</subfield></datafield><datafield tag="520" ind1=" " ind2=" "><subfield code="a">Objective To compare the clinical features, laboratory findings, and prognosis of Behçet’s disease (BD) patients with and without Budd–Chiari syndrome (BCS). Methods This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017. Results Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.005 and p = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p < 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14–47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%). Conclusion To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. 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Budd–Chiari syndrome in Behçet’s disease: a retrospective multicenter study Behçet’s disease (dpeaa)DE-He213 Budd–Chiari syndrome (dpeaa)DE-He213 Inferior vena cava (dpeaa)DE-He213 Prognosis (dpeaa)DE-He213 Thrombosis (dpeaa)DE-He213 |
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Akyol, Lütfi Toz, Bahtiyar Bayındır, Özün Zengin, Orhan Cansu, DöndüÜsküdar Yiğit, Murat Çetin, Gözde Yıldırım Omma, Ahmet Erden, Abdulsamet Küçükşahin, Orhan Altuner, Mehmet Şakir Çorba, BurçinŞeyda Ünal, Ali Uğur Küçük, Hamit Küçük, Adem Balkarli, Ayşe Gönüllü, Emel Tufan, Ayşe Nur Bakırcı, Sibel Öner, Sibel Yılmaz Balcı, Mehmet Ali Kobak, Şenol Yazıcı, Ayten Özgen, Metin Şahin, Ali Koca, Süleyman Serdar Erer, Burak Gül, Ahmet Aksu, Kenan Keser, Gökhan Onat, Ahmet Mesut Kısacık, Bünyamin Kaşifoğlu, Timuçin Çefle, Ayşe Kalyoncu, Umut Sayarlıoğlu, Mehmet |
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budd–chiari syndrome in behçet’s disease: a retrospective multicenter study |
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Budd–Chiari syndrome in Behçet’s disease: a retrospective multicenter study |
abstract |
Objective To compare the clinical features, laboratory findings, and prognosis of Behçet’s disease (BD) patients with and without Budd–Chiari syndrome (BCS). Methods This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017. Results Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.005 and p = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p < 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14–47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%). Conclusion To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. Key Points•Mortality rate is higher in BD-associated BCS patients with IVC involvement.•Chronic and silent form of BD-associated BCS has a better prognosis.•The main treatment options are corticosteroids and immunosuppressive agents, whereas anticoagulant treatment remains controversial. © International League of Associations for Rheumatology (ILAR) 2021 |
abstractGer |
Objective To compare the clinical features, laboratory findings, and prognosis of Behçet’s disease (BD) patients with and without Budd–Chiari syndrome (BCS). Methods This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017. Results Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.005 and p = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p < 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14–47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%). Conclusion To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. Key Points•Mortality rate is higher in BD-associated BCS patients with IVC involvement.•Chronic and silent form of BD-associated BCS has a better prognosis.•The main treatment options are corticosteroids and immunosuppressive agents, whereas anticoagulant treatment remains controversial. © International League of Associations for Rheumatology (ILAR) 2021 |
abstract_unstemmed |
Objective To compare the clinical features, laboratory findings, and prognosis of Behçet’s disease (BD) patients with and without Budd–Chiari syndrome (BCS). Methods This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017. Results Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.005 and p = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p < 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14–47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%). Conclusion To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. Key Points•Mortality rate is higher in BD-associated BCS patients with IVC involvement.•Chronic and silent form of BD-associated BCS has a better prognosis.•The main treatment options are corticosteroids and immunosuppressive agents, whereas anticoagulant treatment remains controversial. © International League of Associations for Rheumatology (ILAR) 2021 |
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