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Int J Clin Exp Pathol 2010;3(6):582-586

Original Article
Autoimmune hepatitis in patients with primary Sjögren’s syndrome: a series of two-
hundred and two patients

Julie K. Karp, Esen K. Akpek, Robert A. Anders

Department of Pathology, The Johns Hopkins Hospital, The Johns Hopkins Hospital, 600 N. Wolfe Street, Pathology Building, Room 401,
Baltimore, MD, USA;  Department of Ophthalmology, Ocular Surface Diseases and Dry Eye Clinic, The Wilmer Eye Institute, The Johns Hopkins
Hospital, 600 N. Wolfe Street, Maumenee 317, Baltimore, MD, USA; Department of Pathology, Division of Gastrointestinal and Liver Pathology,
The Johns Hopkins Hospital, 1550 Orleans Street, Cancer Research Building II, Room 346, Baltimore, MD, USA.

Received June 1, 2010, accepted June 14, 2010, available online June 20, 2010

Abstract: Based on the revised criteria of the American-European Consensus Group, we retrospectively established the diagnosis of primary
or secondary Sjögren's syndrome for 202 patients referred to a Sjögren's syndrome clinic. Of these, 58 patients and 8 patients fulfilled criteria
for primary and secondary Sjögren's syndrome, respectively. Of the 58 patients with primary Sjögren's syndrome, one (1.7%) had definite
autoimmune hepatitis, as defined by the International Autoimmune Hepatitis Group diagnostic criteria. One additional symptomatic patient who
did not fulfill criteria for primary Sjögren's syndrome had definite autoimmune hepatitis.  None of the patients with secondary Sjögren's
syndrome had definite autoimmune hepatitis. Two (1%) of the 194 patients with primary Sjögren's syndrome or clinical symptoms had primary
biliary cirrhosis. These values are lower than those reported by prior studies with smaller patient populations and likely represent a more
accurate estimate of the true prevalence of these diseases in patients with primary Sjögren's syndrome. (IJCEP1006005).

Key words: Autoimmune hepatitis, Sjögren’s syndrome, primary biliary cirrhosis

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Address all correspondence to:
Robert Anders, MD
Department of Pathology
Division of Gastrointestinal and Liver Pathology
The Johns Hopkins Hospital
1550 Orleans Street, Cancer Research Building II
Room 346, Baltimore, MD, 21231, USA.
Tel: 410-955-3511
E-mail:
rander54@jhmi.edu