Biomarkers of lymphoma in Sjögren’s syndrome and evaluation of the lymphoma risk in prelymphomatous conditions: Results of a multicenter study

Quartuccio, Luca; Isola, Miriam; Baldini, Chiara; Priori, Roberta; Bartoloni, Elena; Carubbi, Francesco; Maset, M.; Gregoraci, Giorgia; Mea, Vincenzo Della; Salvin, S.; Marchi, G. De; Luciano, N.; Colafrancesco, Serena; Alunno, Alessia; Giacomelli, Roberto; Gerli, Roberto; Valesini, Guido; Bombardieri, Stefano; Vita, Salvatore De

doi:10.1016/j.jaut.2013.10.002

Cited by 134 publications

(98 citation statements)

References 33 publications

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“…Furthermore, 5% of patients may develop lymphoma, mainly the mucosa-associated lymphoid tissue (MALT) non-Hodgkin lymphoma (NHL), which represents the most severe complication of the disease [2,3]. At present, although several pathogenic mechanisms have been suggested trying to understand the clinical heterogeneity of the disease, several aspects remain unknown.…”

Section: Introductionmentioning

confidence: 99%

Is minor salivary gland biopsy more than a diagnostic tool in primary Sjo¨gren׳s syndrome? Association between clinical, histopathological, and molecular features: A retrospective study

Carubbi

Alunno

Cipriani

et al. 2014

Seminars in Arthritis and Rheumatism

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Section: Introductionmentioning

confidence: 99%

Is minor salivary gland biopsy more than a diagnostic tool in primary Sjo¨gren׳s syndrome? Association between clinical, histopathological, and molecular features: A retrospective study

Carubbi

Alunno

Cipriani

et al. 2014

Seminars in Arthritis and Rheumatism

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“…Some textbooks suggest a role of the disappearance of previously positive autoantibodies as a predictive biomarker for lymphoma development [17]. Several other blood tests, such as anemia [9] or a falling packed-cell volume [6], leucopenia [1,16], neutropenia [8], lymphopenia [1,9], CD4 + T-lymphocytopenia [4,12,17], a high sedimentation rate [9], the presence of anti-SSB/La antibodies [16], and detection of monoclonal rheumatoid factor-associated crossreactive idiotypes (CRI) [12,13] have also been mentioned as risk factors.…”

Section: Open Accessmentioning

confidence: 99%

“…The most described clinical predictor of lymphoma development is an enlargement of the parotid gland [3,6,12,16,19,22]. Also lymphadenopathy and splenomegaly [1,3,6,8,9,12], the presence of skin vasculitis (i.e.…”

Section: Open Accessmentioning

confidence: 99%

“…In SS patients, it is generally known to be the most reliable biochemical predictor of lymphoma development [8,[11][12][13][14][15][16][17] and inferior median survival [8,13,15,18]. Also low complement C3 [4,10,18] and C4 [4,5,8,10,12,[15][16][17][18][19][20] levels have been associated with lymphoma and overall higher mortality rates. The majority of SS patients display a diffuse hypergammaglobulinemia [8,12] and this vigorous expansion makes them probably more susceptible to mutational events [6,12].…”

Section: Introductionmentioning

confidence: 99%

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Prognostic value of cryoglobulins, protein electrophoresis, and serum immunoglobulins for lymphoma development in patients with Sjögren’s syndrome. A retrospective cohort study

Kimman

Bossuyt

Blockmans

2017

Acta Clinica Belgica

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Background:Patients with Sjögren's syndrome (SS) have a considerable higher risk of lymphoma development. Objectives: To determine the incidence of lymphoma and the value of biomarkers to predict lymphoma development in patients with SS. Methods: Clinical files of all patients with a presumed diagnosis of SS between 1991 and 2016 were retrospectively reviewed for the development of lymphoma. Biochemical data were plotted as a function of the relative time before and after the lymphoma diagnosis (for patients who developed lymphoma) or before the last available blood test (for patients who did not develop lymphoma). Correlations between several biochemical parameters and development of lymphoma were analyzed by logistic regression. In order to evaluate the evolution of cryoglobulins, a random effect model with random intercepts was used. Results: Sixteen patients developed a lymphoma (prevalence 8.9%; median follow-up 6 years). Cryoglobulins were significantly higher in these patients (n = 16), when compared to the rest of patients (n = 164) without lymphoma (121 ± 250 versus 8 ± 24.9 mg/L for IgG; 231 ± 422 versus 13 ± 30 mg/L for IgM; 10 ± 20 versus 1 ± 4 mg/L for IgA in the cryoprecipitate). Cryoglobulinlevels were significantly more increasing (p-values for IgG = 0.0007; for IgM = 0.0123; and for IgA in the cryoprecipitate <0.0001) in the time period before the lymphoma diagnosis (patients with lymphoma) compared to the time period before the last available blood test (patients without lymphoma). Also low (i.e. under the detection limit) C3 (OR 13.9) or C4 (OR 7.1) levels, a progressively decreasing total complement activity (OR 6.6), progressively decreasing gammaglobulins (OR 13.4), a persistent detection of monoclonal bands (OR 14.6) on protein electrophoresis, a persistent low or decreasing serum IgG (OR 18), and decreasing IgM-serum levels (OR 17.7) were significantly associated with lymphoma. Conclusion: Periodically follow-up of laboratory markers, such as cryogloblins, over time proved to be an accurate way to predict lymphoma.

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“…15 Moreover, prolonged B-cell survival and aberrant B-cell activity may lead to the development of non-Hodgkin lymphomas (NHL) in 5% of pSS patients; in these cases the extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) represents the most common subtype. 6,11,16 Because of the well-established role of B lymphocytes in the pathogenesis of pSS, a B-cell targeting therapy may represent a new and intriguing therapeutic approach in this disease.…”

Section: Introductionmentioning

confidence: 99%

Rituximab in primary Sjögren's syndrome: a ten-year journey

Carubbi

Alunno

Cipriani

et al. 2014

Lupus

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Primary Sjo¨gren's syndrome (pSS) is an autoimmune disorder affecting exocrine glands and characterized in most cases by a rather mild clinical picture. However, a subgroup of pSS patients experience systemic extraglandular involvement leading to a worsening of disease prognosis. Current therapeutic options for the treatment of pSS are mainly empirical, often translated by other autoimmune diseases, and recent systematic reviews have highlighted the lack of evidence-based recommendations for most of the drugs commonly employed in the spectrum of extraglandular involvement. Because of the well-established role of B-lymphocytes in the pathogenesis of pSS, a B-cell targeting therapy may represent a new and intriguing therapeutic approach; in this context, growing evidence suggests that B-cell depletion by rituximab (RTX) is also effective in pSS. Of interest, besides clinical efficacy, RTX also showed biologic effects, consistently affecting the inflammation and the lymphoid organization that occur in target tissue. Moreover, the good results observed in the published trials after RTX treatment in pSS should represent the starting point to develop evidence-based guidelines for the use of biologic therapy in this disease. Lupus (2014) 23, 1337-1349.

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Biomarkers of lymphoma in Sjögren’s syndrome and evaluation of the lymphoma risk in prelymphomatous conditions: Results of a multicenter study

Cited by 134 publications

References 33 publications

Is minor salivary gland biopsy more than a diagnostic tool in primary Sjo¨gren׳s syndrome? Association between clinical, histopathological, and molecular features: A retrospective study

Is minor salivary gland biopsy more than a diagnostic tool in primary Sjo¨gren׳s syndrome? Association between clinical, histopathological, and molecular features: A retrospective study

Prognostic value of cryoglobulins, protein electrophoresis, and serum immunoglobulins for lymphoma development in patients with Sjögren’s syndrome. A retrospective cohort study

Rituximab in primary Sjögren's syndrome: a ten-year journey

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