scholarly journals Primary Breast Extranodal Marginal Zone Lymphoma in Primary Sjögren Syndrome: Case Presentation and Relevant Literature

2020 ◽  
Vol 9 (12) ◽  
pp. 3997
Author(s):  
Giuseppe Ingravallo ◽  
Eugenio Maiorano ◽  
Marco Moschetta ◽  
Luisa Limongelli ◽  
Mauro Giuseppe Mastropasqua ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Marginal Zone ◽  
Relevant Literature ◽  
Epidemiologic Studies ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Non Hodgkin Lymphoma ◽  
Primary Sjögren Syndrome ◽  
Diagnostic Work ◽  
Work Up

The association between autoimmune diseases, mostly rheumatoid arthritis, systemic lupus erythematosus, celiac disease and Sjögren syndrome, and lymphoma, has been widely demonstrated by several epidemiologic studies. By a mechanism which has not yet been entirely elucidated, chronic activation/stimulation of the immune system, along with the administration of specific treatments, may lead to the onset of different types of lymphoma in such patients. Specifically, patients affected by Sjögren syndrome may develop lymphomas many years after the original diagnosis. Several epidemiologic, hematologic, and histological features may anticipate the progression from Sjögren syndrome into lymphoma but, to the best of our knowledge, a definite pathogenetic mechanism for such progression is still missing. In fact, while the association between Sjögren syndrome and non-Hodgkin lymphoma, mostly extranodal marginal zone lymphomas and, less often, diffuse large B-cell, is well established, many other variables, such as time of onset, gender predilection, sites of occurrence, subtype of lymphoma, and predictive factors, still remain unclear. We report on a rare case of primary breast lymphoma occurring three years after the diagnosis of Sjögren syndrome in a 57-year-old patient. 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.

scholarly journals Primary Breast Extranodal Marginal Zone Lymphoma in Primary Sjögren Syndrome: Case Presentation and Relevant Literature

2020 ◽  
Author(s):  
Giuseppe Ingravallo ◽  
Eugenio Maiorano ◽  
Marco Moschetta ◽  
Luisa Limongelli ◽  
Mauro Giuseppe Mastropasqua ◽  
...  
Keyword(s):  
Marginal Zone ◽  
Relevant Literature ◽  
Epidemiologic Studies ◽  
Original Diagnosis ◽  
Non Hodgkin Lymphoma ◽  
Primary Sjögren Syndrome ◽  
Histological Factors ◽  
Diagnostic Work ◽  
Work Up ◽  
Stimulation Of

The association between autoimmune diseases, mostly rheumatoid arthritis, systemic lupus erythematous, celiac disease and Sjögren syndrome, and lymphoma has been widely demonstrated by several epidemiologic studies. By a not yet entirely elucidated mechanism, chronic activation/stimulation of the immune system, along with the administration of specific treatments, may lead to persistent stimulation of both of B- and T-cells, and to the onset of different types of lymphoma in such patients. Specifically, patients affected by may develop lymphomas may years after the original diagnosis. Several epidemiologic, hematologic and histological factors may anticipate the progression from Sjögren syndrome into lymphoma but, to the best of our knowledge, a definite pathogenetic mechanism for such progression is still missing. In fact, while the association between Sjögren syndrome and non-Hodgkin lymphoma, mostly diffuse large B-cell and extranodal marginal zone lymphomas is well established, many other variables, such as time of onset, gender predilection, sites of occurrence, subtype of lymphoma and predictive factors still remain unclear. We report on a rare case of primary breast lymphoma occurring three years after the diagnosis of Sjögren syndrome in a 57 y.o. patient. The diagnostic work-up, including radiograms, core needle biopsy and histological examination are discussed, along with emerging data from the recent literature, thus highlighting the usefulness of breast surveillance in Sjögren syndrome patients.

Dermatologic Manifestations of Sjögren Syndrome

2011 ◽  
Vol 15 (1) ◽  
pp. 8-14 ◽  
Author(s):  
Ashley Kittridge ◽  
Shannon B. Routhouska ◽  
Neil J. Korman
Keyword(s):  
Lupus Erythematosus ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Dry Eyes ◽  
Cutaneous Manifestations ◽  
Non Hodgkin Lymphoma ◽  
Increased Risk ◽  
Life Threatening ◽  
Lymphome Non Hodgkinien ◽  
Dermatologic Manifestations

Background: Sjögren syndrome (SS) is a chronic autoimmune inflammatory disease that involves primarily the exocrine glands, resulting in their functional impairment. SS typically presents as dry eyes (xerophthalmia) and dry mouth (xerostomia). This process can manifest either as the independent phenomenon of primary SS or as secondary SS when found in the context of another autoimmune process, most commonly rheumatoid arthritis or systemic lupus erythematosus. Nearly half of the patients with SS develop cutaneous manifestations, which may include dry skin (xeroderma), palpable and nonpalpable purpura, and/or urticaria-like lesions. These cutaneous manifestations have been underemphasized because they are often overshadowed by the more prominent sicca symptoms. However, certain skin findings are of paramount clinical and prognostic importance as they confer an increased risk for the development of life-threatening conditions, including multisystem vasculitis and non-Hodgkin lymphoma. Objective and Conclusions: In this review, the cutaneous manifestations of primary SS are discussed, with an emphasis on those findings that portend an increased risk of mortality. Antécédent: Le syndrome de Sjögren (SS) est une maladie autoimmune inflammatoire chronique qui affecte principalement les glandes exocrines, menant à leur incapacité fonctionnelle. SS se caractérise typiquement par une xérophtalmie (sècheresse des yeux) et une xérostomie (bouche sèche). Ces symptômes peuvent apparaître en tant que manifestations indépendantes du SS primaire ou comme SS secondaire, c'est-à-dire comme conséquence d'une autre affection autoimmune, le plus souvent l'arthrite rhumatoïde, ou le lupus érythémateux systémique. Près de la moitié des patients présente des manifestations cutanées de la maladie, dont une peau sèche (xérodermie) et des lésions palpables ou non de purpura ou ressemblant à l'urticaire. Ces manifestations cutanées n'ont pas été suffisamment soulignées car elles sont souvent éclipsées par des symptômes plus notables de la maladie. Toutefois, certaines manifestations cutanées sont d'une grande importance dans le diagnostic clinique et dans le pronostic, car elles représentent une indication d'un risque plus élevé de développer une maladie mortelle telle que la vascularite multisystémique et le lymphome non hodgkinien.

scholarly journals Questions on the Bidirectional Relationship Between Primary Sjögren Syndrome and Non-Hodgkin Lymphoma

2020 ◽  
pp. jrheum.201352
Author(s):  
Yun-Tzu Liang ◽  
Pui-Ying Leong ◽  
James Cheng-Chung Wei
Keyword(s):  
Hodgkin Lymphoma ◽  
Population Based ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Population Based Study ◽  
Bilateral Relationship ◽  
Non Hodgkin Lymphoma ◽  
Primary Sjögren Syndrome ◽  
Bidirectional Relationship

We have read with great interest in the article by Wang, et al on the higher incidence of non-Hodgkin lymphoma (NHL) in patients with primary Sjögren syndrome (pSS) and the higher incidence of pSS in patients with NHL1. Thank you for the discovery of the bilateral relationship between pSS and NHL in this nationwide population-based study1.

scholarly journals PULMONARY NON-HODGKIN LYMPHOMA IN A PRIMARY SJÖGREN SYNDROME PATIENT

2016 ◽  
Vol 25 (2) ◽  
pp. 95-99
Author(s):  
Claudia Deaconu ◽  
◽  
Ruxandra Ionescu ◽  
Daniela Opris ◽  
◽  
...  
Keyword(s):  
Hodgkin Lymphoma ◽  
Membranous Glomerulonephritis ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Syndrome Patient ◽  
Non Hodgkin Lymphoma ◽  
Primary Sjögren Syndrome ◽  
Pulmonary Lymphoma ◽  
Autoimmune Condition ◽  
Lymphocytic Infiltrates

Sjögren syndrome (SS) is a chronic autoimmune condition that mainly targets exocrine glands where it produces lymphocytic infiltrates. The clinical display varies in severity and can include malignancy association such as non-Hodgkin lymphoma. Its incidence ranges from 5 to 8%, while the risk of occurrence is 16 to 44 times higher. This article aims to describe the evolution of a patient with primary SS who developed pulmonary lymphoma for which she received Rituximab as part of the oncological therapeutic scheme. During the course of the disease she developed membranous glomerulonephritis, rarely described in these patients and low complement levels cited as predictive factor for lymphoproliferation.

Increased Frequency of Hand Osteoarthritis in Patients with Primary Sjögren Syndrome Compared with Systemic Lupus Erythematosus

2016 ◽  
Vol 43 (6) ◽  
pp. 1068-1071 ◽  
Author(s):  
Adem Aksoy ◽  
Dilek Solmaz ◽  
Gercek Can ◽  
Pinar Cetin ◽  
Ali Balci ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Daily Practice ◽  
Sjögren Syndrome ◽  
Controlled Study ◽  
Hand Osteoarthritis ◽  
Sjogren Syndrome ◽  
Systemic Lupus ◽  
Primary Sjögren Syndrome ◽  
The Mean

Objective.In daily practice, we noticed that hand osteoarthritis (OA) was commonly associated with primary Sjögren syndrome (pSS). Therefore, we aimed to investigate its prevalence in patients with pSS in a controlled study.Methods.The study included patients with pSS and controls with systemic lupus erythematosus (SLE). Standard hand/wrist radiographs were obtained and classified according to the Kellgren-Lawrence system. “Erosive hand OA” was defined according to the Verbruggen-Veys classification.Results.There were 114 patients with pSS (110 women, 51.0 yrs) and 34 patients with SLE (33 women, 42.4 yrs). Among 114 patients with pSS, 42.7% had radiographic, 30.3% symptomatic, and 16.0% erosive hand OA. The prevalences of radiographic (45.5%) and erosive hand OA (14.4%) in 90 patients with pSS with age- and sex-matched patients with SLE were significantly higher than those in patients with SLE (14.7% and 0.0%, p = 0.007 and p = 0.012, respectively). Interobserver reliabilities for diagnosing radiographic and erosive OA were found to be good (ĸ = 0.780 and ĸ = 0.788, respectively). Intraobserver reliabilities for diagnosing radiographic and erosive OA were also good (ĸ = 0.784 and ĸ = 0.825 for FO, and ĸ = 0.722 and ĸ = 0.800 for AB, respectively). The frequency of hand OA in patients with pSS was found to be increased with increasing age (r = 0.513). The mean age of those with erosive hand OA was significantly higher than those without erosive OA (p < 0.001).Conclusion.This study suggests that pSS, conversely to SLE, is more frequently associated with hand OA.

Bidirectional Relationship Between Primary Sjögren Syndrome and Non-Hodgkin Lymphoma: A Nationwide Taiwanese Population-based Study

2020 ◽  
Vol 47 (9) ◽  
pp. 1374-1378
Author(s):  
Li-Hui Wang ◽  
Wei-Ming Wang ◽  
Chun-Yu Lin ◽  
Sheng-Hsiang Lin ◽  
Chi-Chang Shieh
Keyword(s):  
Hodgkin Lymphoma ◽  
Population Based ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Research Database ◽  
Population Based Study ◽  
Non Hodgkin Lymphoma ◽  
Primary Sjögren Syndrome ◽  
Bidirectional Relationship ◽  
New Onset

Objective.Bidirectional relationships between some autoimmune diseases and non-Hodgkin lymphoma (NHL) may exist. We conducted this nationwide population-based study in Taiwan to investigate whether there is a bidirectional relationship between primary Sjögren syndrome (pSS) and NHL.Methods.Using the National Health Insurance Research Database of Taiwan, we identified 15,636 patients with new-onset pSS without previous cancer and 25,074 patients with new-onset NHL without previous pSS as 2 non-overlapping cohorts from 1998 to 2012, and followed them until 2013. Standardized incidence ratios (SIR) for NHL in the patients with pSS and SIR for pSS in the patients with NHL were compared with the general population.Results.Among the 15,636 patients with pSS, 741 developed cancers, including 51 with NHL. The highest SIR of specific cancer risk in patients with pSS was that for NHL (SIR 4.6, 95% CI 3.4–6.0). Among the 25,074 patients with NHL, 49 developed pSS; the SIR was also increased (SIR 3.2, 95% CI 2.4–4.2). The risk was highest within 1 year after the diagnosis of each disease.Conclusion.This nationwide population-based study is the first to report a bidirectional relationship between pSS and NHL. Our findings suggest being alert for patients with pSS or NHL who have early signs of the other disease in clinical care. The underlying mechanisms of the bidirectional relationship merit further investigation.

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