1.Liver Cirrhosis Progression in a Patient with Overlapping IgG4-Related Sclerosing Cholangitis and Primary Biliary Cholangitis
Dong Wook KIM ; Hee Yoon NOH ; Song-Hee HAN ; Myung Hwan NOH
Korean Journal of Pancreas and Biliary Tract 2025;30(2):87-92
We report a rare case of overlapping immunoglobulin G4-sclerosing cholangitis (IgG4-SC) and primary biliary cholangitis (PBC) in a 78-year-old woman presenting with sudden-onset jaundice, cholestatic liver enzyme elevation, and biliary strictures. Elevated alkaline phosphatase levels, positive antimitochondrial antibodies, increased serum IgG4 levels, and a high IgG4/IgG ratio, combined with endobiliary biopsy via endoscopic retrograde cholangiopancreatography biopsy showing lymphoplasmacytic infiltration and IgG4-positive cells, confirmed the diagnosis. Initial treatment with biliary drainage, ursodeoxycholic acid (UDCA), and corticosteroids provided temporary relief; however, the patient experienced recurrent episodes of cholangitis and biliary obstruction, eventually progressing to decompensated cirrhosis over 2 years. This case highlights the importance of considering IgG4-SC in patients with PBC who fail to respond adequately to UDCA and underscores the significant challenges in diagnosing and managing such overlapping syndromes. Further research is crucial to better understand the underlying pathophysiology, refine therapeutic strategies, and improve clinical outcomes of these rare, complex autoimmune conditions.
2.Liver Cirrhosis Progression in a Patient with Overlapping IgG4-Related Sclerosing Cholangitis and Primary Biliary Cholangitis
Dong Wook KIM ; Hee Yoon NOH ; Song-Hee HAN ; Myung Hwan NOH
Korean Journal of Pancreas and Biliary Tract 2025;30(2):87-92
We report a rare case of overlapping immunoglobulin G4-sclerosing cholangitis (IgG4-SC) and primary biliary cholangitis (PBC) in a 78-year-old woman presenting with sudden-onset jaundice, cholestatic liver enzyme elevation, and biliary strictures. Elevated alkaline phosphatase levels, positive antimitochondrial antibodies, increased serum IgG4 levels, and a high IgG4/IgG ratio, combined with endobiliary biopsy via endoscopic retrograde cholangiopancreatography biopsy showing lymphoplasmacytic infiltration and IgG4-positive cells, confirmed the diagnosis. Initial treatment with biliary drainage, ursodeoxycholic acid (UDCA), and corticosteroids provided temporary relief; however, the patient experienced recurrent episodes of cholangitis and biliary obstruction, eventually progressing to decompensated cirrhosis over 2 years. This case highlights the importance of considering IgG4-SC in patients with PBC who fail to respond adequately to UDCA and underscores the significant challenges in diagnosing and managing such overlapping syndromes. Further research is crucial to better understand the underlying pathophysiology, refine therapeutic strategies, and improve clinical outcomes of these rare, complex autoimmune conditions.
3.Liver Cirrhosis Progression in a Patient with Overlapping IgG4-Related Sclerosing Cholangitis and Primary Biliary Cholangitis
Dong Wook KIM ; Hee Yoon NOH ; Song-Hee HAN ; Myung Hwan NOH
Korean Journal of Pancreas and Biliary Tract 2025;30(2):87-92
We report a rare case of overlapping immunoglobulin G4-sclerosing cholangitis (IgG4-SC) and primary biliary cholangitis (PBC) in a 78-year-old woman presenting with sudden-onset jaundice, cholestatic liver enzyme elevation, and biliary strictures. Elevated alkaline phosphatase levels, positive antimitochondrial antibodies, increased serum IgG4 levels, and a high IgG4/IgG ratio, combined with endobiliary biopsy via endoscopic retrograde cholangiopancreatography biopsy showing lymphoplasmacytic infiltration and IgG4-positive cells, confirmed the diagnosis. Initial treatment with biliary drainage, ursodeoxycholic acid (UDCA), and corticosteroids provided temporary relief; however, the patient experienced recurrent episodes of cholangitis and biliary obstruction, eventually progressing to decompensated cirrhosis over 2 years. This case highlights the importance of considering IgG4-SC in patients with PBC who fail to respond adequately to UDCA and underscores the significant challenges in diagnosing and managing such overlapping syndromes. Further research is crucial to better understand the underlying pathophysiology, refine therapeutic strategies, and improve clinical outcomes of these rare, complex autoimmune conditions.
4.Liver Cirrhosis Progression in a Patient with Overlapping IgG4-Related Sclerosing Cholangitis and Primary Biliary Cholangitis
Dong Wook KIM ; Hee Yoon NOH ; Song-Hee HAN ; Myung Hwan NOH
Korean Journal of Pancreas and Biliary Tract 2025;30(2):87-92
We report a rare case of overlapping immunoglobulin G4-sclerosing cholangitis (IgG4-SC) and primary biliary cholangitis (PBC) in a 78-year-old woman presenting with sudden-onset jaundice, cholestatic liver enzyme elevation, and biliary strictures. Elevated alkaline phosphatase levels, positive antimitochondrial antibodies, increased serum IgG4 levels, and a high IgG4/IgG ratio, combined with endobiliary biopsy via endoscopic retrograde cholangiopancreatography biopsy showing lymphoplasmacytic infiltration and IgG4-positive cells, confirmed the diagnosis. Initial treatment with biliary drainage, ursodeoxycholic acid (UDCA), and corticosteroids provided temporary relief; however, the patient experienced recurrent episodes of cholangitis and biliary obstruction, eventually progressing to decompensated cirrhosis over 2 years. This case highlights the importance of considering IgG4-SC in patients with PBC who fail to respond adequately to UDCA and underscores the significant challenges in diagnosing and managing such overlapping syndromes. Further research is crucial to better understand the underlying pathophysiology, refine therapeutic strategies, and improve clinical outcomes of these rare, complex autoimmune conditions.
5.Liver Cirrhosis Progression in a Patient with Overlapping IgG4-Related Sclerosing Cholangitis and Primary Biliary Cholangitis
Dong Wook KIM ; Hee Yoon NOH ; Song-Hee HAN ; Myung Hwan NOH
Korean Journal of Pancreas and Biliary Tract 2025;30(2):87-92
We report a rare case of overlapping immunoglobulin G4-sclerosing cholangitis (IgG4-SC) and primary biliary cholangitis (PBC) in a 78-year-old woman presenting with sudden-onset jaundice, cholestatic liver enzyme elevation, and biliary strictures. Elevated alkaline phosphatase levels, positive antimitochondrial antibodies, increased serum IgG4 levels, and a high IgG4/IgG ratio, combined with endobiliary biopsy via endoscopic retrograde cholangiopancreatography biopsy showing lymphoplasmacytic infiltration and IgG4-positive cells, confirmed the diagnosis. Initial treatment with biliary drainage, ursodeoxycholic acid (UDCA), and corticosteroids provided temporary relief; however, the patient experienced recurrent episodes of cholangitis and biliary obstruction, eventually progressing to decompensated cirrhosis over 2 years. This case highlights the importance of considering IgG4-SC in patients with PBC who fail to respond adequately to UDCA and underscores the significant challenges in diagnosing and managing such overlapping syndromes. Further research is crucial to better understand the underlying pathophysiology, refine therapeutic strategies, and improve clinical outcomes of these rare, complex autoimmune conditions.
6.Adjuvant Pembrolizumab in Patients with Stage IIIA/N2 Non–Small Cell Lung Cancer Completely Resected after Neoadjuvant Concurrent Chemoradiation: A Prospective, Open-Label, Single-Arm, Phase 2 Trial
Junghoon SHIN ; Sehhoon PARK ; Kyung Hwan KIM ; Eui-Cheol SHIN ; Hyun Ae JUNG ; Jong Ho CHO ; Jong-Mu SUN ; Se-Hoon LEE ; Yong Soo CHOI ; Jin Seok AHN ; Jhingook KIM ; Keunchil PARK ; Young Mog SHIM ; Hong Kwan KIM ; Jae Myoung NOH ; Yong Chan AHN ; Hongryull PYO ; Myung-Ju AHN
Cancer Research and Treatment 2024;56(4):1084-1095
Purpose:
Optimal treatment for stage IIIA/N2 non–small cell lung cancer (NSCLC) is controversial. We aimed to assess the efficacy and safety of adjuvant pembrolizumab for stage IIIA/N2 NSCLC completely resected after neoadjuvant concurrent chemoradiation therapy (CCRT).
Materials and Methods:
In this open-label, single-center, single-arm phase 2 trial, patients with stage IIIA/N2 NSCLC received adjuvant pembrolizumab for up to 2 years after complete resection following neoadjuvant CCRT. The primary endpoint was disease-free survival (DFS). Secondary endpoints included overall survival (OS) and safety. As an exploratory biomarker analysis, we evaluated the proliferative response of blood CD39+PD-1+CD8+ T cells using fold changes in the percentage of proliferating Ki-67+ cells from days 1 to 7 of cycle 1 (Ki-67D7/D1).
Results:
Between October 2017 and October 2018, 37 patients were enrolled. Twelve (32%) and three (8%) patients harbored EGFR and ALK alterations, respectively. Of 34 patients with programmed cell death ligand 1 assessment, 21 (62%), nine (26%), and four (12%) had a tumor proportion score of < 1%, 1%-50%, and ≥ 50%, respectively. The median follow-up was 71 months. The median DFS was 22.4 months in the overall population, with a 5-year DFS rate of 29%. The OS rate was 86% at 2 years and 76% at 5 years. Patients with tumor recurrence within 6 months had a significantly lower Ki-67D7/D1 among CD39+PD-1+CD8+ T cells than those without (p=0.036). No new safety signals were identified.
Conclusion
Adjuvant pembrolizumab may offer durable disease control in a subset of stage IIIA/N2 NSCLC patients after neoadjuvant CCRT and surgery.
7.Changes in the Long-term Prognosis of Crohn’s Disease between 1986 and 2015: The Population-Based Songpa-Kangdong Inflammatory Bowel Disease Cohort Study
Byong Duk YE ; Sung Noh HONG ; Seung In SEO ; Ye-Jee KIM ; Jae Myung CHA ; Kyoung Hoon RHEE ; Hyuk YOON ; Young-Ho KIM ; Kyung Ho KIM ; Sun Yong PARK ; Seung Kyu JEONG ; Ji Hyun LEE ; Hyunju PARK ; Joo Sung KIM ; Jong Pil IM ; Sung Hoon KIM ; Jisun JANG ; Jeong Hwan KIM ; Seong O SUH ; Young Kyun KIM ; Sang Hyoung PARK ; Suk-Kyun YANG ; On behalf of the Songpa-Kangdong Inflammatory Bowel Disease (SKIBD) Study Group
Gut and Liver 2022;16(2):216-227
Background/Aims:
The long-term course of Crohn’s disease (CD) has never been evaluated in non-Caucasian population-based cohorts. The aim of the present study was to evaluate the longterm prognosis of Korean CD patients in the well-defined population-based Songpa-Kangdong inflammatory bowel disease cohort.
Methods:
Outcomes of disease and their predictors were evaluated for 418 patients diagnosed with CD between 1986 and 2015.
Results:
During a median of 123 months, systemic corticosteroids, thiopurines, and anti-tumor necrosis factor (TNF) agents were administered to 58.6%, 81.3%, and 37.1% of patients, respectively. Over time, the cumulative probability of starting corticosteroids significantly decreased (p=0.001), whereas that of starting thiopurines and anti-TNFs significantly increased (both p<0.001). The cumulative probability of behavioral progression was 54.5% at 20 years, and it significantly decreased during the anti-TNF era. Intestinal resection was required for 113 patients (27.0%). The cumulative probabilities of intestinal resection at 1, 5, 10, 20, and 25 years after CD diagnosis were 12.7%, 16.5%, 23.8%, 45.1%, and 51.2%, respectively. Multivariable Cox regression analysis identified stricturing behavior at diagnosis (adjusted hazard ratio [aHR], 2.70; 95% confidence interval [CI], 1.55 to 4.71), penetrating behavior at diagnosis (aHR, 11.15; 95% CI, 6.91 to 17.97), and diagnosis of CD during the anti-TNF era (aHR, 0.51; 95% CI, 0.35 to 0.76) as independently associated with intestinal resection. The standardized mortality ratio among CD patients was 1.36 (95% CI, 0.59 to 2.68).
Conclusions
The long-term prognosis of Korean patients with CD is at least as good as that of Western CD patients, as indicated by the low intestinal resection rate. Moreover, behavioral progression and intestinal resection rates have decreased over the past 3 decades.
8.Clinical Characteristics and Long-term Prognosis of Elderly-Onset Ulcerative Colitis in a Population-Based Cohort in the SongpaKangdong District of Seoul, Korea
Sang Hyoung PARK ; Seung Kyu JEONG ; Ji Hyun LEE ; Kyoung Hoon RHEE ; Young-Ho KIM ; Sung Noh HONG ; Kyung Ho KIM ; Seung In SEO ; Jae Myung CHA ; Sun Yong PARK ; Hyunju PARK ; Joo Sung KIM ; Jong Pil IM ; Hyuk YOON ; Sung Hoon KIM ; Jisun JANG ; Jeong Hwan KIM ; Seong O SUH ; Young Kyun KIM ; Byong Duk YE ; Suk-Kyun YANG ;
Gut and Liver 2021;15(5):742-751
Background/Aims:
We aimed to evaluate the clinical characteristics and long-term prognosis of elderly-onset ulcerative colitis (EOUC) in Korean patients over a 30-year period using a wellestablished population-based cohort in the Songpa-Kangdong district of Seoul, Korea.
Methods:
Clinical characteristics and prognosis were compared between two groups: EOUC,defined as UC diagnosed in individuals aged ≥60 years and non-EOUC (N-EOUC), defined asUC diagnosed in individuals aged 18 to 59 years.
Results:
We identified 99 patients with EOUC (10.3%) and 866 patients with N-EOUC (89.7%) between 1986 and 2015. During the median follow-up of 104.5 months, the overall exposure tomedications was comparable between patients with EOUC and N-EOUC (p=0.091 for corticosteroids, p=0.794 for thiopurines, and p=0.095 for anti-tumor necrosis factor agents). The cumula-tive risks of disease outcomes were also comparable between patients with EOUC and N-EOUC (22.4% vs 30.4% for proximal disease extension [p=0.351], 11.9% vs 18.1% for hospitalization [p=0.240], and 2.3% vs 1.8% for colectomy [p=0.977]) at 10 years after diagnosis. Multivariate Cox regression analysis revealed that corticosteroid use at diagnosis was an independent predic-tor of proximal disease extension (hazard ratio [HR], 6.216; 95% confidence interval [CI], 1.314 to 28.826) and hospitalization (HR, 11.241; 95% CI, 3.027 to 41.742) in patients with EOUC.
Conclusions
In this population-based study from Korea, the pattern of medication use seemed comparable between the EOUC and N-EOUC groups. Moreover, patients with EOUC and those with N-EOUC have a similar disease course in terms of proximal disease extension, hospitaliza-tion, and colectomy.
9.Clinical Characteristics and Long-term Prognosis of Elderly-Onset Ulcerative Colitis in a Population-Based Cohort in the SongpaKangdong District of Seoul, Korea
Sang Hyoung PARK ; Seung Kyu JEONG ; Ji Hyun LEE ; Kyoung Hoon RHEE ; Young-Ho KIM ; Sung Noh HONG ; Kyung Ho KIM ; Seung In SEO ; Jae Myung CHA ; Sun Yong PARK ; Hyunju PARK ; Joo Sung KIM ; Jong Pil IM ; Hyuk YOON ; Sung Hoon KIM ; Jisun JANG ; Jeong Hwan KIM ; Seong O SUH ; Young Kyun KIM ; Byong Duk YE ; Suk-Kyun YANG ;
Gut and Liver 2021;15(5):742-751
Background/Aims:
We aimed to evaluate the clinical characteristics and long-term prognosis of elderly-onset ulcerative colitis (EOUC) in Korean patients over a 30-year period using a wellestablished population-based cohort in the Songpa-Kangdong district of Seoul, Korea.
Methods:
Clinical characteristics and prognosis were compared between two groups: EOUC,defined as UC diagnosed in individuals aged ≥60 years and non-EOUC (N-EOUC), defined asUC diagnosed in individuals aged 18 to 59 years.
Results:
We identified 99 patients with EOUC (10.3%) and 866 patients with N-EOUC (89.7%) between 1986 and 2015. During the median follow-up of 104.5 months, the overall exposure tomedications was comparable between patients with EOUC and N-EOUC (p=0.091 for corticosteroids, p=0.794 for thiopurines, and p=0.095 for anti-tumor necrosis factor agents). The cumula-tive risks of disease outcomes were also comparable between patients with EOUC and N-EOUC (22.4% vs 30.4% for proximal disease extension [p=0.351], 11.9% vs 18.1% for hospitalization [p=0.240], and 2.3% vs 1.8% for colectomy [p=0.977]) at 10 years after diagnosis. Multivariate Cox regression analysis revealed that corticosteroid use at diagnosis was an independent predic-tor of proximal disease extension (hazard ratio [HR], 6.216; 95% confidence interval [CI], 1.314 to 28.826) and hospitalization (HR, 11.241; 95% CI, 3.027 to 41.742) in patients with EOUC.
Conclusions
In this population-based study from Korea, the pattern of medication use seemed comparable between the EOUC and N-EOUC groups. Moreover, patients with EOUC and those with N-EOUC have a similar disease course in terms of proximal disease extension, hospitaliza-tion, and colectomy.
10.Acute Acalculous Cholecystitis Associated with Sunitinib Treatment for Renal Cell Carcinoma
Se Woong CHOI ; Jeong Min LEE ; Dong Gyun KIM ; Myung Hwan NOH
The Korean Journal of Gastroenterology 2020;75(2):103-107
A 64-year-old man was treated with sunitinib as a first-line therapy for metastatic renal cell carcinoma. He was given oral sunitinib in cycles of 50 mg once daily for 2 weeks followed by a week off. During the 5th week of treatment right upper quadrant pain developed, but this resolved spontaneously during the 6th week (off treatment). However, on the 8th week of treatment, he was admitted to hospital because the acute right upper quadrant pain recurred with nausea, vomiting, and fever. Acute acalculous cholecystitis was then diagnosed by ultrasonography and CT. In addition, his laboratory findings indicated disseminated intravascular coagulation. Accordingly, sunitinib therapy was discontinued and broad-spectrum antibiotics initiated. He subsequently recovered after emergent percutaneous cholecystostomy. His Naranjo Adverse Drug Reaction Probability Scale score was 7, indicaing a probable association of the event with sunitinib. Suspicion of sunitinib-related acute cholecystitis is required, because, although uncommon, it can be life-threatening.
Acalculous Cholecystitis
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Anti-Bacterial Agents
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Carcinoma, Renal Cell
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Cholecystitis, Acute
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Cholecystostomy
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Disseminated Intravascular Coagulation
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Drug-Related Side Effects and Adverse Reactions
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Fever
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Humans
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Middle Aged
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Nausea
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Ultrasonography
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Vomiting