scholarly journals Sjögren syndrome diagnosis in a cohort of patients with breast cancer: a single-center experience

2021 ◽  
Vol 3 (2) ◽  
Author(s):  
Nadia Melillo ◽  
Matteo Landriscina ◽  
Antonello Trotta ◽  
Stefania Sciacca ◽  
Francesco Paolo Cantatore

The association between estrogen receptor (ER) positive breast cancer (BC) and autoimmune disorders has been recently recognized. In particular exposure to aromatase inhibitors is associated with a significant increased risk of rheumatological autoimmune disorders. The purpose of this study was to investigate Sjögren syndrome (SjS) occurrence in patients with ER-positive BC. This is a prospective study analyzing 110 consecutive patients with ER-positive BC treated with anti-hormonal therapy. New 2016 American College of Rheumatology/European League against Rheumatism (ACR-EULAR) classification criteria were used to identify patients with SjS. Ultrasonography of salivary glands (SG) was used to screen patients with negative disease biomarkers, to candidate them to SGs biopsy. Sicca syndrome was detected in 51 patients (46%), whereas a true primary SjS was diagnosed in 11 patients (10%). Even if the evaluation of incidence and prevalence of primary SjS vary widely, to the best of our knowledge, the data from the present study emphasize a previously unsuspected high prevalence of defined pSjS that causes BC sicca symptoms complaints. Hypothesis, explanation of this link and even possible biases are discussed.

2021 ◽  
pp. jrheum.201175
Author(s):  
Robert I. Fox ◽  
Carla M. Fox

Jarrot and colleagues1 present a retrospective evaluation of 148 patients with primary Sjögren syndrome (SS) who fulfilled the American College of Rheumatology/European League Against Rheumatism criteria for SS, and also fulfilled the criteria for spondyloarthritis (SpA). The patients were largely from Marseille, France, and were followed over a prolonged time period.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Pei-I Kuo ◽  
Tzu-Min Lin ◽  
Yu-Sheng Chang ◽  
Tsung-Yun Hou ◽  
Hui-Ching Hsu ◽  
...  

AbstractThe risk of bisphosphonate-related osteonecrosis of the jaw (BRONJ) in primary Sjogren syndrome (pSS) has rarely been explored. To explore the association between BRONJ and pSS, we conducted a population-based propensity-score-matched cohort study using Taiwan’s National Health Insurance Research Database, including pSS patients receiving antiosteoporotic therapy and patients without pSS receiving antiosteoporotic therapy. A 1:4 matched-pair cohort based on propensity score was created. The stratified Cox proportional hazards model compared the risk of BRONJ in the pSS and non-pSS groups. In the study, 23,280 pSS patients and 28,712,152 controls were enrolled. After matching, 348 patients with pSS receiving antiosteoporotic drugs and 50,145 without pSS receiving antiosteoporotic drugs were included for analysis. The risk of developing BRONJ was 1.96 times higher in pSS patients compared with non-pSS patients after adjustment for age, sex, and comorbidities. No dose–response effect was observed in the bisphosphonate-treated pSS cohorts, documented as the cumulative defined daily doses of either < 224 or ≥ 224 (hazard ratio [HR]: 2.407, 95% confidence interval [CI] 1.412–7.790; HR: 2.143, 95% CI 1.046–4.393, respectively) increased risk of developing osteonecrosis of the jaw. In conclusion, the risk of BRONJ is significantly higher in patients with pSS compared with the general population.


1997 ◽  
Vol 15 (4) ◽  
pp. 1385-1394 ◽  
Author(s):  

PURPOSE Adjuvant tamoxifen has been shown to reduce relapse and mortality among node-positive post-menopausal breast cancer patients. The value of adding chemotherapy to tamoxifen is controversial. PATIENTS AND METHODS Between July 1986 and April 1993, 1,266 postmenopausal breast cancer patients with node-positive disease were randomly assigned to receive one of four adjuvant therapy regimens: (A) tamoxifen alone for 5 years; (B) tamoxifen plus three courses of early cyclophosphamide, methotrexate, and fluorouracil (CMF) on months 1, 2, and 3; (C) tamoxifen plus delayed single courses of CMF on months 9, 12, and 15; (D) tamoxifen plus early and delayed CMF on months 1, 2, 3, 9, 12, and 15. The two-by-two factorial design allowed two direct comparisons: early CMF (B and D) versus no early CMF (A and C), and delayed CMF (C and D) versus no delayed CMF (A and B). Estrogen receptor (ER) status was known for all patients and was used to stratify the randomization. A total of 1, 212 patients (96%) were eligible and assessable. The median follow-up duration was 60 months. RESULTS The results of the two-by-two factorial comparisons were as follows: (1) early CMF added to tamoxifen significantly improved 5-year disease-free survival (DFS; 64% v 57%; hazards ratio [HR], 0.79; 95% confidence interval [CI], 0.66 to 0.95; P = .01); and (2) delayed CMF added to tamoxifen did not improve DFS (5-year DFS, 61% v 60%; HR, 0.97; 95% CI, 0.81 to 1.17; P = .77). For patients with ER-positive tumors, the addition of CMF, either early or delayed or both, reduced the relative risk of relapse by 22% to 36%. In contrast, for patients with ER-negative tumors, tamoxifen with delayed CMF was associated with a nonsignificant increased risk of relapse (HR, 1.27; 95% CI, 0.92 to 1.76; P = .15). CONCLUSION Postmenopausal patients with node-positive breast cancer should be offered combination chemotherapy in addition to tamoxifen. Tamoxifen should not be initiated before CMF, as this might be detrimental, especially for patients with ER-negative tumors.


2011 ◽  
Vol 15 (1) ◽  
pp. 8-14 ◽  
Author(s):  
Ashley Kittridge ◽  
Shannon B. Routhouska ◽  
Neil J. Korman

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.


2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Rasika Ranaweerage ◽  
Shehan Perera ◽  
Aruna Gunapala

Abstract Background Acquired Gitelman syndrome is a very rare disorder reported in association with autoimmune disorders, mostly Sjögren syndrome. It is characterized by the presence of hypokalaemic metabolic alkalosis, hypocalciuria, hypomagnesaemia and hyper-reninaemia, in the absence of typical genetic mutations associated with inherited Gitelman syndrome. Case presentation A 20 year old woman who was previously diagnosed with primary Sjögren syndrome and autoimmune thyroiditis presented with two week history of lower limb weakness and salt craving. Examination revealed upper limb and lower limb muscle weakness with muscle power of 3/5 on MRC scale and diminished deep tendon reflexes. On evaluation, she had hypokalaemia with high trans-tubular potassium gradient, metabolic alkalosis and hypocalciuria, features suggestive of Gitelman syndrome. New onset hypokalaemic alkalosis in a previously normokalaemic patient with Sjögren syndrome strongly favored a diagnosis of acquired Gitelman syndrome. Daily potassium supplementation and spironolactone resulted in complete clinical recovery. Conclusions Acquired Gitelman syndrome associated with Sjögren syndrome is rare. It should be considered as a differential diagnosis during evaluation of acute paralysis and hypokalaemic metabolic alkalosis in patients with autoimmune disorders, especially Sjögren syndrome.


2017 ◽  
Vol 44 (8) ◽  
pp. 1184-1189 ◽  
Author(s):  
J. Antonio Aviña-Zubieta ◽  
Michael Jansz ◽  
Eric C. Sayre ◽  
Hyon K. Choi

Objective.To estimate the future risk and time trends of venous thromboembolism (VTE) in individuals with newly diagnosed primary Sjögren syndrome (pSS) in the general population.Methods.Using a population database that includes all residents of British Columbia, Canada, we created a study cohort of all patients with incident SS and up to 10 controls from the general population matched for age, sex, and entry time. We compared incidence rates (IR) of pulmonary embolism (PE), deep vein thrombosis (DVT), and VTE between the 2 groups according to SS disease duration. We calculated HR, adjusting for confounders.Results.Among 1175 incident pSS cases (mean age 56.7 yrs, 87.6% women), the IR of PE, DVT, and VTE were 3.9, 2.8, and 5.2 per 1000 person-years (PY), respectively; the corresponding rates in the comparison cohort were 0.9, 0.8, and 1.4 per 1000 PY. Compared with non-SS individuals, the multivariable HR for PE, DVT, and VTE among SS cases were 4.07 (95% CI 2.04–8.09), 2.80 (95% CI 1.27–6.17), and 2.92 (95% CI 1.66–5.16), respectively. The HR matched for age, sex, and entry time for VTE, PE, and DVT were highest during the first year after SS diagnosis (8.29, 95% CI 2.57–26.77; 4.72, 95% CI 1.13–19.73; and 7.34, 95% CI 2.80–19.25, respectively).Conclusion.These findings provide population-based evidence that patients with pSS have a substantially increased risk of VTE, especially within the first year after SS diagnosis. Further research into the involvement of monitoring and prevention of VTE in SS may be warranted.


2018 ◽  
Vol 5 (1) ◽  
pp. 18
Author(s):  
Noemie Tremblay ◽  
Alexandra Bourque ◽  
Jean-Pascal Costa

We report a case of an 82-year-old woman diagnosed with Sjögren syndrome on the basis of salivary gland biopsy and anti-Ro/SSA antibodies who presented to our institution with a pruriginous maculopapular pancorporeal rash with diffuse eosinophilic infiltrate and mild eosinophilia. Sicca symptoms were mild. Cutaneous and hematologic findings resolved spontaneously within 4 weeks, the rash did not respond to antihistamines but was mildly attenuated by topical corticosteroid. Both the rash and hypereosinophilia greatly improved while the patient received oral corticosteroids for a contrast medium allergy before a CT-scan. When the patient was diagnosed with SS, hydroxychloroquine treatment was introduced. This case report aims to raise awareness of the possibility of such a clinical presentation, considering the known increased risk of non-hodgkin lymphoma associated with Sjögren syndrome estimated to 5%-10% lifetime risk which is 5 to 44 times higher than that of the general population.


Sign in / Sign up

Export Citation Format

Share Document