PSC with elevated serum IgG4

[Guest guest]

Dear All;

The following paper was just published online in the American

Journal of Gastroenterology, and suggests that the small fraction of

PSC patients with elevated serum IgG4 might (?) benefit from

coricosteroids, similar to those patients with autoimmune

pancreatitis:

Mendes, Flavia D., nsen, a, Keach, Jill, Katzmann, Jerry

A., Smyrk, , Donlinger, , Chari, Suresh & Lindor,

D.

Elevated Serum IgG4 Concentration in Patients with Primary

Sclerosing Cholangitis.

The American Journal of Gastroenterology [in Press] (2006)

OBJECTIVES: Biliary strictures, similar to primary sclerosing

cholangitis (PSC), have been reported in patients with autoimmune

pancreatitis, which is characterized by elevated serum IgG4 levels

and responsiveness to corticosteroids. We sought to determine the

frequency of elevated IgG4 in patients with PSC and to clinically

compare PSC patients with elevated and normal IgG4 levels.

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.

Best regards,

Dave

(father of (21); PSC 07/03; UC 08/03)