Malignant lymphoma in primary Sjögren's syndrome: an update on the pathogenesis and treatment.

OBJECTIVES: Sjögren's syndrome (SS), a chronic autoimmune disorder, particularly compromises the function of exocrine glands. Its association with lymphoma is well documented. Our aim was to systematically review the molecular, clinical, histopathologic, and therapeutic aspects of these SS-related malignant lymphoproliferations. METHODS: The literature was searched for original articles published between 1968 and 2012 focusing on the risk factors for lymphoma development in Sjögren's syndrome using MEDLINE and PubMed. The search terms we used were "Sjögren's syndrome," "lymphoma," and "risk factors." All papers identified were English-language, full-text papers. RESULTS: A low-grade marginal-zone lymphoma related to mucosa-associated lymphoid tissue is the commonest lymphoid neoplasia in SS. The majority of SS-associated lymphomas are characterized by localized stage, indolent clinical course, and recurrence in other extranodal sites. Although the transition from a chronic inflammatory condition to malignant lymphoma is a multistep process that is yet poorly understood, there is increasing evidence that chronic antigenic stimulation by an exoantigen or autoantigens plays an essential role in the development of SS-associated lymphoproliferation. CONCLUSIONS: This review discusses the pathogenetic aspects of lymphomagenesis in SS. Recent advances in the treatment of lymphoma in SS are also stated.

Autopathogenic correlation of periodontitis and rheumatoid arthritis.

Recently, a number of studies have pointed to a potential relationship between periodontitis (PO) and RA and vice versa. Both diseases are characterized by chronic inflammation, osseous destruction, damage of the supporting soft tissues, similar cellular immune responses and common immunogenetic findings. Although a definite, methodological report associating these diseases is missing from the literature, it is possible that both diseases share a common aetiopathogenic background. This background includes the post-translation modification citrullination, which guides the conversion of the amino acid arginine to citrulline in certain self-proteins, generating neo-epitope structures. This results in reduced self-tolerance, development of autoimmunity and the production of ACPAs. The current hypothesis suggests that certain oral bacteria induce the citrullination of proteins under the action of the enzyme peptidyl arginine deiminase (PAD), which exists in both Porphyromonas gingivalis and inflammatory cells. Antibodies against citrullinated proteins and peptides constitute a common serological finding in both RA and PO. The aim of this review is to map the immunological and serological profiles of PO, and to unveil the parameters that connect PO with the appearance of RA at clinical, prognostic and pathogenetic levels. Until now, there have been no reports sufficiently mapping the immunological profile of PO and defining its aetiopathogenic connection with RA, although a similarity between the immunological profile of PO and RA is highly expected.