PACK syndrome: A case series and review

Collins, Bradford S.; Dillon, D; Silver, Richard M.

doi:10.1016/j.amjms.2022.12.005

Cited by 2 publications

(1 citation statement)

References 38 publications

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“…In 1971, Reynolds et al reported six patients with PBC and CREST syndrome. Although many cases have been reported since then, the etiology and outcome of this combined disorder remain largely unknown (7)(8)(9)(10)(11)(12)(13)(14) .…”

Section: Introductionmentioning

confidence: 99%

Evaluation of GLOBE and UK-PBC Scores and Long- Term Outcomes in Primary Biliary Cholangitis Complicated with CREST Syndrome

Abe,

Hayashi,

Sugaya

et al. 2023

Preprint

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Aim Primary biliary cholangitis (PBC) is frequently associated with autoimmune disease. Although there have been some reports of PBC complicated with CREST syndrome (PBC-CREST), the long-term prognosis of these patients has not been fully investigated. Herein, the long-term prognosis of PBC-CREST was compared with that of PBC alone using GLOBE and UK-PBC scores. Methods A total of 302 patients diagnosed with PBC between December 1990 and August 2021 at our hospital and related institutions were included. The survival rates without liver transplantation (LT) were compared between patients with PBC-CREST (n = 57) and those with PBC alone (n = 245). Moreover, 173 patients were divided into two groups (PBC-CREST (n = 26) and PBC alone (n = 147)), excluding those with LT/liver-related death within 1 year after ursodeoxycholic acid administration; GLOBE and UK-PBC scores were compared. Results The survival rates without LT (3/5/10 years) were 98%/96%/96% for the PBC-CREST group and 92%/87%/80% for the PBC-alone group, with a significantly better prognosis in the PBC-CREST group (log-rank, P = 0.0172). The predicted liver-related death and LT risk (5/10/15 years) based on the UK-PBC score was significantly lower in the PBC-CREST group (2.4%/7.6%/13.2%) than in the PBC-alone group (4.8%/11.8%/18.8%; P < 0.05). The predicted LT-free survival (3/5 years) based on the GLOBE score was significantly higher in the PBC-CREST group (93%/88%) than in the PBC-alone group (88%/81%; P < 0.05). Multivariate analysis revealed that the presence of CREST syndrome is an independent protective factor for the presence of cirrhosis. Conclusions PBC-CREST may have a better long-term prognosis than PBC alone.

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Section: Introductionmentioning

confidence: 99%

Evaluation of GLOBE and UK-PBC Scores and Long- Term Outcomes in Primary Biliary Cholangitis Complicated with CREST Syndrome

Abe,

Hayashi,

Sugaya

et al. 2023

Preprint

View full text Add to dashboard Cite

show abstract

Long-term outcomes in patients with primary biliary cholangitis complicated with CREST syndrome

Abe,

Hayashi,

Sugaya

et al. 2024

Sci Rep

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Primary biliary cholangitis (PBC) is frequently associated with autoimmune disease. Although PBC complicated with CREST syndrome (PBC-CREST) has been reported, the long-term outcomes of the affected patients have not been fully investigated. Herein, the long-term outcomes of PBC-CREST were evaluated. Next, the GLOBE and UK-PBC scores were validated and compared between the PBC alone and PBC-CREST groups. A total of 302 patients who were diagnosed with PBC between December 1990 and August 2021 at Fukushima Medical University Hospital were included. The liver transplantation (LT)-free survival rates were compared between patients with PBC alone (n = 245) and those with PBC-CREST (n = 57). Moreover, 173 patients, excluding those with liver-related death/LT within 1 year after ursodeoxycholic acid administration, were divided into two subgroups (PBC alone (n = 147) and PBC-CREST (n = 26)), and the GLOBE and UK-PBC scores were compared between the subgroups. The survival rates without LT (3/5/10 years) were 92/87/80% for the PBC-alone group and 98/96/96% for the PBC-CREST group, with a significantly better prognosis in the PBC-CREST group (log-rank P = 0.0172). Multivariate analysis revealed that the presence of CREST syndrome is an independent protective factor for the presence of cirrhosis. The predicted 5/10/15-year risks of liver-related death or LT based on the UK-PBC score were significantly lower in the PBC-CREST group (2.4/7.6/13.2%) than in the PBC-alone group (4.8/11.8/18.8%) (P < 0.05). The predicted 3/5-year LT-free survival rates based on the GLOBE score were significantly higher in the PBC-CREST group (93/88%) than in the PBC-alone group (88/81%) (P < 0.05). Patients with PBC-CREST may have better long-term outcomes than those with PBC alone.

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PACK syndrome: A case series and review

Cited by 2 publications

References 38 publications

Evaluation of GLOBE and UK-PBC Scores and Long- Term Outcomes in Primary Biliary Cholangitis Complicated with CREST Syndrome

Evaluation of GLOBE and UK-PBC Scores and Long- Term Outcomes in Primary Biliary Cholangitis Complicated with CREST Syndrome

Long-term outcomes in patients with primary biliary cholangitis complicated with CREST syndrome

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