Lymphoma and other malignancies in primary Sjogren's syndrome: a cohort study on cancer incidence and lymphoma predictors

Theander, Elke; Henriksson, Gunnel; Ljungberg, Otto; Mandl, Thomas; Manthorpe, R; Jacobsson, Lennart

doi:10.1136/ard.2005.041186

Cited by 502 publications

(456 citation statements)

References 41 publications

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“…A retrospective study of Swedish patients with "non-AECC" sicca syndrome did not observe an increased risk of cancer (standardized incidence ratio [95% confidence interval]: 0.77 [0.41-1.32]). Interestingly, neither acute leukaemia nor lymphoma was diagnosed [11].…”

Section: Discussionmentioning

confidence: 99%

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Unusual association of two cases of acute myeloblastic leukaemia and possible Sjogren’s syndrome and review of literature

Brault¹,

Gourguechon²,

Chehimi³

et al. 2017

Hematol Leuk

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Section: Discussionmentioning

confidence: 99%

“…Although many studies have focused on the risk of neoplasia in SS [10][11][12][13][14], we found only four publications in which acute leukaemia was cited ( Table 1) [15][16][17][18]. The first publication reported on a 59-year-old woman with primary SS diagnosed in 1963.…”

Section: Literature Reviewmentioning

confidence: 99%

Unusual association of two cases of acute myeloblastic leukaemia and possible Sjogren’s syndrome and review of literature

Brault¹,

Gourguechon²,

Chehimi³

et al. 2017

Hematol Leuk

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“…The patients are also at higher risk of developing lymphoma, a well-identified cause of mortality in pSS. [Ramos-Casals et al 2005;Theander et al 2004Theander et al , 2006.…”

Section: Treatment Of Other Glandular Manifestationsmentioning

confidence: 99%

“…The systemic nonexocrine manifestations may include constitutional symptoms (fatigue, arthralgia, myalgia and low-grade fever), arthritis, skin lesions, leukocytoclastic vasculitis, Raynaud's phenomenon, central and peripheral neurologic involvement, renal, lung or hepatic involvement and finally lymphoproliferative disorders [Ramos-Casals et al 2005]. The presence of vasculitis, cryoglobulinaemia and low complement levels characterizes a subset of patient with higher morbidity and mortality mainly related to the higher probability to develop lymphoma [Ramos-Casals et al 2005;Theander et al 2004Theander et al , 2006. SS may occur as a primary disorder (pSS) or in association with other systemic autoimmune diseases, traditionally defined as secondary SS (sSS), such as rheumatoid arthritis, and systemic lupus erythematosus [Ramos-Casals et al 2007].…”

mentioning

confidence: 99%

“…During the past few years, a growing body of evidence has induced clinicians to distinguish two different variants of the syndrome: an exocrine gland-localized disease, that mainly affects quality of life, and a systemic syndrome, which is characterized by extra-glandular manifestations, and may potentially evolve to lymphoma [Ramos-Casals et al 2005;Theander et al 2004Theander et al , 2006. Whereas symptomatic therapy may be enough for most of patients with limited glandular disease, the lack of disease-modifying drugs has an important impact for those patients with systemic manifestations and severe organ involvement.…”

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confidence: 99%

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Treating Sjögren’s syndrome: insights for the clinician

Vitali¹,

Palombi²,

Cataleta³

2010

Therapeutic Advances in Musculoskeletal

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Sjögren's syndrome (SS) is a systemic autoimmune disease that affects the exocrine glands, mainly the salivary and lachrymal glands, with consequent persistent dryness of the mouth and eyes. In addition to the clinical manifestations related to the exocrine gland involvement, a consistent prevalence of patients may present systemic manifestations. Some of these can be ascribed to the periepithelial extension of lymphocytic infiltration whilst others are determined by an immunomediated process affecting small-or medium-size vessels. While the use of tear and saliva substitutes and local or systemic stimulation of residual secretions represent the mainstays of the therapy of sicca component, different immunomodulating or immunosuppressive agents are usually required to treat extraglandular features, similarly to what happens in other connective tissue diseases. In the last few years, the advancement in the understanding the pathogenetic mechanisms of this disorder and the availability of new biologic target therapies seem to offer completely new therapeutic options. The use of B cell depleting or modulating therapies has achieved promising results.

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Chronic Immune Stimulation and Subsequent Waldenström Macroglobulinemia

Koshiol

Gridley²,

Engels³

et al. 2008

Arch Intern Med

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Background Certain autoimmune and infectious conditions are associated with increased risks of subtypes of non-Hodgkin’s lymphomas (NHL). A few prior studies suggest that chronic inflammation may particularly elevate risk for the distinct NHL subtype Waldenström’s macroglobulinemia (WM). We assessed WM risk in relation to a wide range of chronic immune stimulatory conditions among 4 million U.S. veterans. Methods We identified 361 WM cases with up to 27 years of follow-up. Using time-dependent Poisson regression we estimated rate ratios (RR) and 95% confidence intervals (CI) for WM risk in relation to history of autoimmune diseases that typically have autoantibodies (with systematic or organ involvement) or do not have autoantibodies, infections, and allergies. All models were adjusted for attained age, calendar-year, race, number of hospital visits, and latency between study entry and exit. Results The age-standardized incidence of WM was 0.34/100,000 person-years. WM risk was elevated among individuals with any prior autoimmune condition (RR, 2.2; 95% CI, 1.7–3.0), autoantibodies with systemic involvement (RR, 2.50; 95% CI, 1.55–4.02), autoantibodies with organ involvement (RR, 2.30; 95% CI, 1.57–3.37). Risks for WM were also increased with hepatitis (RR, 3.39; 95% CI, 1.38–8.30), human immunodeficiency virus (HIV) (RR, 12.05; 95% CI, 2.83–51.46), and rickettsiosis (RR, 3.35; 95% CI, 1.38–8.14) Conclusions In the largest investigation of WM risk factors to date, we found 2- to 3-fold elevated risk of WM among persons with a personal history of autoimmune diseases with autoantibodies and notably elevated risks for hepatitis, HIV, and rickettsiosis. Our findings provide novel insights into the as yet unknown etiology of WM.

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Lymphoma and other malignancies in primary Sjogren's syndrome: a cohort study on cancer incidence and lymphoma predictors

Abstract: A 16-fold increased risk for development of NHL was found. CD4+ T lymphocytopenia is an additional strong risk factor for developing lymphoma.

Cited by 502 publications

References 41 publications

Unusual association of two cases of acute myeloblastic leukaemia and possible Sjogren’s syndrome and review of literature

Unusual association of two cases of acute myeloblastic leukaemia and possible Sjogren’s syndrome and review of literature

Treating Sjögren’s syndrome: insights for the clinician

Chronic Immune Stimulation and Subsequent Waldenström Macroglobulinemia

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