Abstract | OBJECTIVES: METHODS: We measured serum IgG4 in 127 patients with PSC and 87 patients with primary biliary cirrhosis, as disease controls. Demographic, clinical, and laboratory characteristics were compared between the PSC groups with normal and elevated IgG4 (>140 mg/dL). RESULTS: Elevated IgG4 was found in 12 PSC patients (9%) versus one PBC patient (1.1%) (p= 0.017). Patients with elevated IgG4 had higher total bilirubin (p= 0.009), alkaline phosphatase (p= 0.01), and PSC Mayo risk score (p= 0.038), and lower frequency of IBD (p < 0.0001). Importantly, the time to liver transplantation was shorter in patients with elevated IgG4 (1.7 vs 6.5 yr, p= 0.0009). The type of biliary involvement (intrahepatic, extrahepatic, or both) and pancreatic involvement were similar in both groups. CONCLUSIONS: A small proportion of PSC patients had elevated serum IgG4. In these patients parameters of liver disease severity were more pronounced and time to liver transplantation was shorter, suggesting a more severe disease course. It is possible that this subset of patients behaves similarly to autoimmune pancreatitis patients with biliary strictures, and could potentially respond to corticosteroids. Testing PSC patients for IgG4 and treating those with elevated levels with corticosteroids in clinical trials should be considered.
|
Authors | Flavia D Mendes, Roberta Jorgensen, Jill Keach, Jerry A Katzmann, Thomas Smyrk, Jessica Donlinger, Suresh Chari, Keith D Lindor |
Journal | The American journal of gastroenterology
(Am J Gastroenterol)
Vol. 101
Issue 9
Pg. 2070-5
(Sep 2006)
ISSN: 0002-9270 [Print] United States |
PMID | 16879434
(Publication Type: Comparative Study, Journal Article)
|
Chemical References |
- Biomarkers
- Immunoglobulin G
- Alkaline Phosphatase
- Bilirubin
|
Topics |
- Adult
- Aged
- Alkaline Phosphatase
(blood)
- Bilirubin
(blood)
- Biomarkers
(blood)
- Biopsy
- Cholangitis, Sclerosing
(blood, pathology, surgery)
- Disease Progression
- Female
- Follow-Up Studies
- Humans
- Immunoglobulin G
(blood)
- Liver Transplantation
- Male
- Middle Aged
- Nephelometry and Turbidimetry
- Prognosis
- Retrospective Studies
- Risk Factors
|