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We report the case of a patient with primary sclerosing cholangitis associated with Sjögren's syndrome and Raynaud's phenomenon, an uncommon association. The presence of periportal lymph nodes with features similar to those described in autoimmune diseases suggests an immune pathogenesis of the disease. The localized and segmentary involvement of the common duct is also an uncommon finding. The pathogenetic and therapeutic aspects are discussed.
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