Clinical, Immunologic, and Molecular Factors Predicting Lymphoma Development in Sjogren’s Syndrome Patients

  title={Clinical, Immunologic, and Molecular Factors Predicting Lymphoma Development in Sjogren’s Syndrome Patients},
  author={Michael Voulgarelis and Fotini Skopouli},
  journal={Clinical Reviews in Allergy \& Immunology},
Among autoimmune diseases, Sjogren’s syndrome (SS) displays the highest incidence of non-Hodgkin lymphoma (NHL) development with the salivary extranodal marginal zone B cell lymphomas being the most common type. The majority of SS-associated NHLs 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 yet poorly understood, there is… 
Predicting lymphoma development in patients with Sjögren’s syndrome
ABSTRACT Introduction: The issue of predicting lymphoma in primary Sjögren’s syndrome (pSS) starts from its clinical and biologic essence, i.e., an autoimmune exocrinopathy with sicca syndrome,
Possible Mechanisms of Lymphoma Development in Sjögren’s Syndrome
Primary Sjögren’s syndrome (pSS) is a systemic as well as an organ-specific autoimmune disease characterized by lymphocytic infiltration of the glandular epithelial tissue. SS patients have been
Controversies on Rituximab Therapy in Sjögren Syndrome-Associated Lymphoproliferation
Rituximab (RTX), a chimeric monoclonal antibody targeting the CD20 antigen on the B cell surface, has been successfully investigated in autoimmune diseases, such as rheumatoid arthritis, systemic lupus erythematosus, ANCA-associated vasculitis, and mixed cryoglobulinemic syndrome.
Primary Breast Extranodal Marginal Zone Lymphoma in Primary Sjögren Syndrome: Case Presentation and Relevant Literature
The diagnostic work-up, including radiograms, core needle biopsy, and histological examination, is discussed, along with emerging data from the recent literature, thus highlighting the usefulness of breast surveillance in Sjögren syndrome patients.
Absence of somatic MYD88 L265P mutations in patients with primary Sjogren’s syndrome
The absence of the MyD88 L265P somatic mutation in the SS cohort does not exclude a common germline susceptibility gene in SS, but it might suggest a distinct operating pathogenetic mechanism in SS-related lymphoma compared with WM and other hematological malignancies.
Lymphoproliferative Disorders Associated with Sjögren Syndrome
The prognosis and outcome of non-Hodgkin lymphoma following Sjogren syndrome will be summarized, and possible predictors and mechanisms of the development will be reviewed.
Determinants of disease severity in primary Sjögren’s syndrome
Several clinical, laboratory and histologic markers can be used to estimate disease prognosis of pSS patients, and the EULAR Sjogren’s Syndrome disease activity index (ESSDAI) is a novel European score to assess disease activity which has not been tested in clinical practice, yet.
Peripheral T-cell lymphoma that developed during the follow-up of IgG4-related disease.
The first case of NHL is described, compatible with a subtype of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), which arose in IgG4-related sclerosing cholangitis.
Identification of lymphoma predictors in patients with primary Sjögren’s syndrome: a systematic literature review and meta-analysis
Lymphadenopathy, parotid enlargement, palpable purpura, low C4 serum levels and cryoglobulins were the most consistent non-Hodgkin´s lymphoma/lymphoproliferative disease predictors.


Clinicopathological factors relating malignant lymphoma with Sjögren's syndrome.
The spectrum of benign to malignant lymphoproliferation in Sjögren's syndrome.
In Sjogren's syndrome, as in these other conditions, it seems likely that a combination of genetic, immunologic, and viral or other unknown environmental factors plays a role in pathogenesis.
Lymphomas in patients with Sjögren's syndrome: review of the literature and physiopathologic hypothesis.
An hypothesis on the physiopathology of lymphoproliferation in patients with SS is made and it is made that, in both diseases, the first event of lymphomagenesis is the chronic stimulation of polyclonal B-cells secreting rheumatoid factor (RF).
Salivary gland lymphomas in patients with Sjögren's syndrome may frequently develop from rheumatoid factor B cells.
The main finding was that the Ig products of the neoplastic B cells were rheumatoid factors (RF).
Lymphoma development in Sjögren's syndrome: novel p53 mutations.
The findings indicate a probable role for the DNA damage response genes in the pathogenesis of this syndrome and implicate dysregulation of this tumor suppressor gene as a possible mechanism for lymphoma development in SS.
Characterization of prelymphomatous stages of B cell lymphoproliferation in Sjögren's syndrome.
The different types of B cell clonal expansion may imply a different risk of lymphoma progression and an accurate clinical, histopathologic, and molecular characterization may be crucial in future studies aimed at clarifying the pathobiology of SS-associated lymphoproliferation.
Predictors of lymphoma development in primary Sjögren's syndrome.
Malignant lymphoma in primary Sjögren's syndrome: a multicenter, retrospective, clinical study by the European Concerted Action on Sjögren's Syndrome.
The novel observations of this study were those related to the type of MNHL, the survival prognosis, and the very high frequency of skin vasculitis, peripheral nerve involvement, anemia, and lymphopenia.
Expression of a cross-reactive idiotype on rheumatoid factor in patients with Sjogren's syndrome.
It is demonstrated that RF from 12/15 SS patients contains a cross-reactive idiotype (CRI) on their kappa light chain defined by a monoclonal antibody (MoAb 17-109) and immunoblotting.