Abstract

Borrowing from the carcinogenesis field, the natural history of Sjögrens syndrome (SS) is analyzed from an evolutionary and multistage point of view. SS is divided into three stages called initiation, promotion and progression. The first stage may be asymptomatic and characterized only by serum autoantibodies (e.g. anti-Ro). The second stage is clinical autoimmune exocrinopathy with potential for lymphocyte aggressive behavior, extraglandular tissue infiltration and pseudolymphoma. The final stage is malignant lymphoma. This sequence is compared with the natural history of human immunodeficiency virus infection which also progresses from an asymptomatic state through AIDS-related complex to AIDS. Indeed, some HIV-infected patients have developed a clinical picture resembling SS due to salivary gland lymphoid infiltrates. This relatively new finding suggests a possible viral etiology for SS.

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