We report a case of AILD and sicca syndrome. The patient had presented with renal insufficiency, lymphadenopathy, hepatosplenomegaly, polyclonal hypergammaglobulinaemia, dryness of the eyes and mouth. Lip biopsy specimens showed an unusual cellular infiltrate similar to his kidney lesions. Data from the eight previously reported cases support the hypothesis that the association is a distinct pathological entity differing from pseudolymphoma and malignant lymphoma, which occur in the course of Sjögren's syndrome. The recognition of AILD is important because lymphoproliferation may lead to death after a few months.
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