PSC Clinical Epidemiology in China

Study Overview
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Brief Summary
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Primary sclerosing cholangitis (PSC) is a rare disease but is increasingly reported in China (mainly in the Chinese language). However, most of the PSC literatures reported from China are case reports, small case series, and review articles. Up to now, there is no information on the epidemiology and disease burden of PSC in China. This study would use EMR/HIS and research databases to investigate the epidemiology, cascade, and treatment pattern of PSC in China.

Detailed Description
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Identify the PSC cases diagnosed from the earliest date available to the present in the SuValue Research Database and the HIS/EMR from clinical sites through diagnostic ICD codes, laboratory, pathology, imaging, endoscopy, surgery, medication, hospitalization, and health outcomes. Collect information on PSC cases' key demographic, the time of PSC diagnosis, hematological/biochemical variables (ALT, AST, ALP, GGT, ALP TBil, ALB), MRCP or ERCP or pathological report, IBD (UC or CD) symptoms, or colon, medication information, clinical outcomes (including cirrhosis, decompensated cirrhosis, death or liver transplantation) as well as current or past treatment.

Official Title
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Primary Sclerosing Cholangitis in China: Epidemiology, Cascade and Treatment

Conditions
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Primary Sclerosing Cholangitis

Intervention / Treatment
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* Other: observation


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Retrospectively review PSC cases before April 1, 2002 will be identified by diagnostic codes from the International Classification of Diseases (ICD)-9, and for those after April 1, 2002, we used ICD-10. We identified patients using an electronic search of SuValue Research Database and HIS of BFH-CMU and BYH-CMU using PSC related ICD codes (ICD-9: 576.1; ICD-10: K83.016/K83.016(China version)). To supplement the ICD search, an electronic keyword search for primary sclerosing cholangitis or seclerosing cholangitis was conducted on all radiology, endoscopy, pathology reports beginning on earliest date available in the database of SuValue Research Database and HIS of BFH-CMU and BYH-CMU. We reviewed the medical charts of identified patients and diagnostic criteria including cholestatic biochemistry (ALP, GGT elevations), and typical imaging (MRCP or ERCP) or pathological features, excluding other known etiology. For patients with multiple visits for PSC, we reviewed only the index visit. To ensure consistency, chart review was independently assessed by two hepatologists. Discrepancies in compliance were resolved by discussion between the two main reviewers. Any unresolved discrepancies were decided by a third expert hepatologist. Exclusion Criteria: The cases with secondary sclerosing cholangitis caused by cholelithiasis, IgG4 related disease, malignancy or other known etiologies. The cases with missing key results on clinical, biochemical (ALP, GGT, bilirubin), radiological (MRCP or ERCP) or histological investigations.
Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Intervention/Treatment |
| --- |
|Other: observation|This observational study does not have any intervention.|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| The prevalence of PSC | The crude prevalence of PSC patients | 7 years | 
| Crowd Distribution of PSC in China | Crowd distribution includes the age-specified/sex-specified distribution | 7 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| The positive rate of ANCA | The percentage of patients with anti-neutrophil cytoplasmic antibodies (ANCA) positive | 0 to 7 years | 
| Dynamic changes of aminotransferase | Dynamic changes of alanine aminotransferase (ALT) from year-0 to year-7 | 0 to 7 years | 
| Dynamic changes of aspartate aminotransferase | Dynamic changes of aspartate aminotransferase (AST) from year-0 to year-7 | 0 to 7 years | 
| Dynamic changes of alkaline phosphatase | Dynamic changes of alkaline phosphatase (ALP) from year-0 to year-7 | 0 to 7 years | 
| Dynamic changes of gamma-glutamyl transpeptidase | Dynamic changes of gamma-glutamyl transpeptidase (GGT) from year-0 to year-7 | 0 to 7 years | 
| Dynamic changes of serum total bilirubin | Dynamic changes of serum total bilirubin (TBIL) from year-0 to year-7 | 0 to 7 years | 
| Dynamic changes of the radiological features measured by Magnetic Resonance Cholangiopancreatography (MRCP) or Encoscopic Retrograde Cholangio-Pancreatography (ERCP) | Dynamic changes of the radiological features at baseline and after 1, 3, 5, 7 years of treatment | 0 to 7 years | 
| Incidence of concomitant diseases | Concomitant diseases include IBD (UC or CD), AIH, PBC, cirrhosis, cholelithiasis or cholangiocarcinoma | 0 to 7 years | 
| Cumulative incidence of clinical outcomes | Cumulative incidence of liver decompensation (including ascites, hepatic encephalopathy, esophageal varices bleeding and Hepatocellular Carcinoma) , liver transplantation, cholangiocarcinoma and death | 0 to 7 years | 
| The medication information | Document medication information from year-0 to year-7 | 0 to 7 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Epidemiology, Cascade, Treatment