SANDRA GOFINET PASOTO

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Instituto Central, Hospital das Clínicas, Faculdade de Medicina - Médico
LIM/17 - Laboratório de Investigação em Reumatologia, Hospital das Clínicas, Faculdade de Medicina
LIM/03 - Laboratório de Medicina Laboratorial, Hospital das Clínicas, Faculdade de Medicina

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  • article 51 Citação(ões) na Scopus
    Systemic manifestations of primary Sjogren's syndrome out of the ESSDAI classification: prevalence and clinical relevance in a large international, multi-ethnic cohort of patients
    (2019) RETAMOZO, S.; ACAR-DENIZLI, N.; RASMUSSEN, A.; HORVATH, I. F.; BALDINI, C.; PRIORI, R.; SANDHYA, P.; HERNANDEZ-MOLINA, G.; ARMAGAN, B.; PRAPROTNIK, S.; KVARNSTROM, M.; GERLI, R.; SEBASTIAN, A.; SOLANS, R.; RISCHMUELLER, M.; PASOTO, S. G.; VALIM, V.; NORDMARK, G.; KRUIZE, A. A.; NAKAMURA, H.; HOFAUER, B.; GIACOMELLI, R.; TREVISANI, V. Fernandes Moca; DEVAUCHELLE-PENSEC, V.; ATZENI, F.; GHEITA, T. A.; CONSANI-FERNANDEZ, S.; SZANTO, A.; SIVILS, K.; GATTAMELATA, A.; DANDA, D.; KILIC, L.; BARTOLONI, E.; BOMBARDIERI, S.; SANCHEZ-GUERRERO, J.; WAHREN-HERLENIUS, M.; MARIETTE, X.; RAMOS-CASALS, M.; BRITO-ZERON, P.
    Objectives: To analyse the frequency and characterise the systemic presentation of primary Sjogren's syndrome (SS) out of the ESSDAI classification in a large international, multi-ethnic cohort of patients. Methods: The Big Data Sjogren Project Consortium is an international, multicentre registry based on world-wide data-sharing and cooperative merging of pre-existing clinical SS databases from leading centres in clinical research in SS from the five continents. A list of 26 organ-by-organ systemic features not currently included in the ESSDAI classification was defined according to previous studies; these features were retrospectively recorded. Results: Information about non-ESSDAI features was available in 6331 patients [5,917 female, mean age at diagnosis 52 years, mainly White (86.3%)]. A total of 1641 (26%) patients had at least one of the ESSDAI systemic features. Cardiovascular manifestations were the most frequent organ-specific group of non-ESSDAI features reported in our patients (17% of the total cohort), with Raynaud's phenomenon being reported in 15%. Patients with systemic disease due to non-ESSDAI features had a lower frequency of dry mouth (90.7% vs. 94.1%, p<0.001) and positive minor salivary gland biopsy (86.7% vs. 89%, p=0.033), a higher frequency of anti-Ro/SSA (74.7% vs. 68.7%, p<0.001), anti-La/SSB antibodies (44.5% vs. 40.4%, p=0.004), ANA (82.7% vs. 79.5%, p=0.006), low C3 levels (17.4% vs. 9.7%, p<0.001), low C4 levels (14.4% vs. 9.6%, p<0.001), and positive serum cryoglobulins (8.6% vs. 5.5%, p=0.001). Systemic activity measured by the ESSDAI, clinESSDAI and DAS was higher in patients with systemic disease out of the ESSDAI in comparison with those without these features (p<0.001 for all comparisons). Conclusions: More than a quarter of patients with primary SS may have systemic manifestations not currently included in the ESSDAI classification, with a wide variety of cardiovascular, digestive, pulmonary, neurological, ocular, ENT (ear, nose, and throat), cutaneous and urological features that increase the scope of the systemic phenotype of the disease. However, the individual frequency of each of these non-ESSDAI features was very low, except for Raynaud's phenomenon.
  • article 2 Citação(ões) na Scopus
    Anti-DNase I Antibody A New Serological Reactivity in Primary Sjogren Syndrome
    (2020) GRIFFO, Priscilla; VIANA, Vilma V. S. T.; PASOTO, Sandra G.; LEON, Elaine P.; BONFA, Eloisa
    Background and Objective: Primary Sjogren syndrome (pSS) is a systemic autoimmune rheumatic disease that particularly affects exocrine glands. Dry eye is one of the most important features of this syndrome, and a recent study reported reduced deoxyribonuclease I (DNase I) activity in the tear of patients with dry eye. We therefore postulated that patients with pSS might have antibodies targeting DNAse I. Methods: We have evaluated in a cross-sectional study 85 patients with pSS (2002 American-European Consensus Group Criteria), 50 rheumatoid arthritis (RA) patients (1987 American College of Rheumatology Criteria) without sicca symptoms, and 88 healthy volunteers. IgG anti-DNase I was detected by enzyme-linked immunosorbent assay using as antigen bovine pancreas enzyme and confirmed by immunoblotting. Results: Age and sex were alike in the 3 groups (p > 0.05). Anti-DNase I was detected in 43.5% of the pSS patients. In contrast, this reactivity was absent in all RA patients (p = 0.0001). Additional comparison of pSS patients with (n = 37) or without (n = 48) anti-DNase I showed that the former group had higher IgG serum levels (2293.2 +/- 666.2 vs 1483.9 +/- 384.6 mg/dL, p = 0.0001) and greater rate of non-drug-induced leukopenia (43% vs 19%, p = 0.02). A multivariate logistic regression analysis identified that only IgG levels were independently associated with anti-DNase I. Conclusions: We describe a high frequency of anti-DNase I antibodies in pSS patients associated with higher serum IgG levels. The lack of this reactivity in RA patients without sicca symptoms suggests that this antibody may be helpful in the differential diagnosis of these diseases.
  • article 0 Citação(ões) na Scopus
    Quality of life, fatigue, sleep quality, and mental health in systemic lupus erythematosus patients with a high cardiovascular risk profile
    (2023) MAZZOLANI, Bruna Caruso; SMAIRA, Fabiana Infante; SIECZKOWSKA, Sofia; ROMERO, Marina; RIBEIRO, Thaina Toledo; AMARANTE, Milla Cordeiro; PASOTO, Sandra; PINTO, Ana Lucia de Sa; LIMA, Fernanda Rodrigues; BENATTI, Fabiana Braga; BONFA, Eloisa; ROSCHEL, Hamilton; GUALANO, Bruno
    Systemic lupus erythematosus (SLE) patients report worse health-related quality of life (HRQL), fatigue, anxiety, depression, and sleep quality, when compared to the general population and other chronic diseases. Furthermore, cardiometabolic diseases are highly prevalent in SLE and are also associated with these parameters. Thus, it is plausible to suggest that SLE patients with a high cardiovascular risk may report worse results for these parameters. The aim of the study is to describe HRQL, fatigue, anxiety and depression symptoms, and sleep quality in a sample of SLE patients with a high cardiovascular risk profile (i.e., BMI between 25 and 40 kg/m2 and/or dyslipidemia, hypertension, or diabetes). This was a cross-sectional study where patients were assessed for (i) demographic, anthropometric, and disease-related parameters, (ii) HRQL, (iii) fatigue, (iv) anxiety and depression symptoms, and (v) sleep quality. One-hundred patients completed the study; however, only 87 patients were assessed for sleep quality data. Patients averaged 41.7 & PLUSMN; 9 years, and most patients were classified as overweight/obese (87%). SF-36 scores for physical and mental components summary were 51.3 & PLUSMN; 9.6 and 54.2 & PLUSMN; 15.6, respectively, with ""bodily pain"" and ""role emotional"" presenting the lower scores. The total SLEQOL score was 105.1 & PLUSMN; 42.0, with lower scores reported for ""self-image"" and ""mood."" Fatigue score was 30.8 & PLUSMN; 8.9, and 78% and 93% reported severe symptoms of anxiety and depression, respectively. The average sleep effectiveness was 82.9 & PLUSMN; 6.6%. Sleep latency, total time in bed (TTiB), and total sleep time (TST) were 8.4 & PLUSMN; 8.9, 495.8 & PLUSMN; 79.7, and 409.7 & PLUSMN; 69.9 min, respectively. Patients reported an average of 17.8 & PLUSMN; 6.2 WE, with 4.5 & PLUSMN; 1.5 min duration and a WASO of 77.7 & PLUSMN; 36.6 min. Despite similar HRQL, fatigue, and sleep quality parameters to those reported by other SLE populations, SLE patients with a high cardiovascular risk had a higher prevalence of depression and anxiety. Understanding SLE patients' quality of life and psychological symptoms is of utmost importance to improve disease management. The findings of this study highlight the need for more intensive and global care regarding mental health when considering a high cardiovascular risk in SLE.
  • article 61 Citação(ões) na Scopus
    Epidemiological profile and north-south gradient driving baseline systemic involvement of primary Sjogren's syndrome
    (2020) BRITO-ZERON, Pilar; ACAR-DENIZLI, Nihan; NG, Wan-Fai; HORVATH, Ildiko Fanny; RASMUSSEN, Astrid; SEROR, Raphaele; LI, Xiaomei; BALDINI, Chiara; GOTTENBERG, Jacques-Eric; DANDA, Debashish; QUARTUCCIO, Luca; PRIORI, Roberta; HERNANDEZ-MOLINA, Gabriela; ARMAGAN, Berkan; KRUIZE, Aike A.; KWOK, Seung-Ki; KVARNSTROM, Marika; PRAPROTNIK, Sonja; SENE, Damien; GERLI, Roberto; SOLANS, Roser; RISCHMUELLER, Maureen; MANDL, Thomas; SUZUKI, Yasunori; ISENBERG, David; VALIM, Valeria; WILAND, Piotr; NORDMARK, Gunnel; FRAILE, Guadalupe; BOOTSMA, Hendrika; NAKAMURA, Hideki; GIACOMELLI, Roberto; DEVAUCHELLE-PENSEC, Valerie; HOFAUER, Benedikt; BOMBARDIERI, Michele; TREVISANI, Virginia Fernandes Moca; HAMMENFORS, Daniel; PASOTO, Sandra G.; RETAMOZO, Soledad; GHEITA, Tamer A.; ATZENI, Fabiola; MOREL, Jacques; VOLLENWEIDER, Cristina; ZEHER, Margit; SIVILS, Kathy; XU, Bei; BOMBARDIERI, Stefano; SANDHYA, Pulukool; VITA, Salvatore De; MINNITI, Antonina; SANCHEZ-GUERRERO, Jorge; KILIC, Levent; HEIJDEN, Eefje van der; PARK, Sung-Hwan; WAHREN-HERLENIUS, Marie; MARIETTE, Xavier; RAMOS-CASALS, Manuel
    Objective. To characterize the systemic phenotype of primary Sjogren's syndrome at diagnosis by analysing the EULAR-SS disease activity index (ESSDAI) scores. Methods. The Sjogren Big Data Consortium is an international, multicentre registry based on worldwide data-sharing cooperative merging of pre-existing databases from leading centres in clinical research in Sjogren's syndrome from the five continents. Results. The cohort included 10 007 patients (9352 female, mean 53 years) with recorded ESSDAI scores available. At diagnosis, the mean total ESSDAI score was 6.1; 81.8% of patients had systemic activity (ESSDAI score >= 1). Males had a higher mean ESSDAI (8.1 vs 6.0, P < 0.001) compared with females, as did patients diagnosed at <35 years (6.7 vs 5.6 in patients diagnosed at >65 years, P < 0.001). The highest global ESSDAI score was reported in Black/African Americans, followed by White, Asian and Hispanic patients (6.7, 6.5, 5.4 and 4.8, respectively; P < 0.001). The frequency of involvement of each systemic organ also differed between ethnic groups, with Black/African American patients showing the highest frequencies in the lymphadenopathy, articular, peripheral nervous system, CNS and biological domains, White patients in the glandular, cutaneous and muscular domains, Asian patients in the pulmonary, renal and haematological domains and Hispanic patients in the constitutional domain. Systemic activity measured by the ESSDAI, clinical ESSDAI (clinESSDAI) and disease activity states was higher in patients from southern countries (P < 0.001). Conclusion. The systemic phenotype of primary Sjogren's syndrome is strongly influenced by personal determinants such as age, gender, ethnicity and place of residence, which are key geoepidemiological players in driving the expression of systemic disease at diagnosis.
  • article 31 Citação(ões) na Scopus
    EBV reactivation serological profile in primary Sjogren's syndrome: an underlying trigger of active articular involvement?
    (2013) PASOTO, Sandra Gofinet; NATALINO, Renato Romera; CHAKKOUR, Henrique Pires; VIANA, Vilma dos Santos Trindade; BUENO, Cleonice; LEON, Elaine Pires; VENDRAMINI, Margarete Borges Gualhardo; LEVY NETO, Mauricio; BONFA, Eloisa
    Antibody to Epstein-Barr virus (EBV) early antigen diffuse (anti-EA-D) is associated with viral replication. However, their possible associations with clinical/therapeutic features in primary Sjogren's syndrome (pSS) were not established. We evaluated 100 pSS patients (American-European Criteria) and 89 age/gender/ethnicity-matched healthy controls. Disease activity was measured by EULAR Sjogren's Syndrome Disease Activity Index (ESSDAI). Antibodies to EBV (anti-VCA IgG/IgM, anti-EBNA-1 IgG, anti-EA-D IgG) were determined by ELISA. Patients and controls had comparable frequencies and mean levels of anti-VCA IgG (90 vs. 86.5 %, p = 0.501; 2.6 +/- A 1.1 vs. 2.5 +/- A 1.1 AU/mL, p = 0.737) and anti-EBNA-1 IgG (92 vs. 94.4 %, p = 0.576; 141.3 +/- A 69.8 vs. 135.6 +/- A 67.5 RU/mL, p = 0.464). Anti-VCA IgM was negative in all cases. Noteworthy, higher frequency and increased mean levels of anti-EA-D were observed in patients than controls (36 vs. 4.5 %, p < 0.0001; 38.6 +/- A 57.4 vs. 7.9 +/- A 26.3 RU/mL, p < 0.0001). Further analysis of patients with (n = 36) and without (n = 64) anti-EA-D revealed comparable age/gender/ethnicity (p a parts per thousand yen 0.551), current prednisone dose (4.8 +/- A 6.9 vs. 5.1 +/- A 10.4 mg/day, p = 0.319), and current uses of prednisone (52.8 vs. 37.5 %, p = 0.148) and immunosuppressants (44.4 vs. 31.3 %, p = 0.201). ESSDAI values were comparable (p = 0.102), but joint activity was more frequent (25 vs. 9.4 %, p = 0.045) in anti-EA-D positive patients. Anti-EA-D antibodies were not associated with anti-Ro/SSA (p = 1.000), anti-La/SSB (p = 0.652), rheumatoid factor (p = 1.000), anti-alpha-fodrin (p = 0.390) or antiphospholipid antibodies (p = 0.573), not suggesting cross-reactivity. The higher anti-EA-D frequency associated with joint activity raises the possibility that a subclinical EBV reactivation may trigger or perpetuate the articular involvement in pSS.
  • article 13 Citação(ões) na Scopus
    Clinical and laboratory features of African-Brazilian patients with systemic sclerosis
    (2020) MENDES, Cristiane; VIANA, Vilma S. T.; PASOTO, Sandra G.; LEON, Elaine P.; BONFA, Eloisa; SAMPAIO-BARROS, Percival D.
    Objective African-Brazilians comprise a group of blacks and ""pardos."" As racial differences can be associated with distinct presentations, we evaluated the clinical and serological associations of African-Brazilians with systemic sclerosis (SSc). Methods Sera from 260 adult SSc patients (203 whites and 57 African-Brazilians) were evaluated. Patients with overlap syndromes were excluded. Clinical and demographic data were obtained from an electronic register database. Laboratory analysis included the following: anti-CENP-A/CENP-B, Scl70, RNA polymerase III, Ku, fibrillarin, Th/To, PM-Scl75, and PM-Scl100 by line immunoassay and anti-nuclear antibodies (ANA) by indirect immunofluorescence (IIF) on HEp-2 cells. Results African-Brazilian SSc patients presented shorter disease duration (12.8 +/- 6.5 vs. 15.9 +/- 8.1 years, p = 0.009), higher frequency of nucleolar ANA pattern (28% vs. 13%, p = 0.008), and lower frequencies of centromeric ANA pattern (14% vs. 29%, p = 0.026) and CENP-B (18% vs. 34%, p = 0.017), as well as an association with severe interstitial lung disease (58% vs. 43%; p = 0.044). Further comparison of ethnic groups according to subsets revealed that diffuse SSc African-Brazilian patients presented higher frequency of pulmonary hypertension (p = 0.017), heart involvement (p = 0.037), nucleolar ANA pattern (p = 0.036), anti-fibrillarin antibodies (p = 0.037), and higher mortality (48% vs. 19%; p = 0.009). A different pattern was observed for the limited subset with solely a lower frequency of esophageal involvement (p = 0.050) and centromeric ANA pattern (p = 0.049). Survival analysis showed that African-Brazilians had a higher mortality, when adjusted for age, gender, and clinical subset (RR 2.06, CI 95% 1.10-3.83, p = 0.023). Conclusion African-Brazilians have distinct characteristics according to clinical subset and an overall more severe SSc than whites, similar to the blacks from other countries.
  • article 2 Citação(ões) na Scopus
    Primary dental care treatment in primary Sjogren's syndrome: a possible role in improving salivary flow rate
    (2022) MARTINS, V. A. O.; FLORIANO, T. F.; LEON, E. P.; VILLAMARIN, L. E. B.; DEVEZA, G. B. H.; AIKAWA, N. E.; SILVA, C. A. A.; KUPA, L. V. K.; PERES, M. P. S. M.; BRAZ-SILVA, P. H.; BONFA, E.; PASOTO, S. G.
    Objective Primary Sjogren's syndrome (pSS) is an inflammatory chronic disorder that mainly affects exocrine glands. Additionally, oral infections can aggravate the glandular dysfunction. However, data on primary dental care (PDC) treatment in pSS are scarce. This study aimed to appraise the impact of PDC on the Xerostomia Inventory (XI), unstimulated/stimulated salivary flow rates and salivary cytokine profile in pSS. Methods Fifty-two pSS patients and 52 sex- and age-matched control participants without systemic autoimmune diseases were included in a prospective study. At inclusion, all participants were assessed through a standardised protocol, measurement of salivary pro-inflammatory cytokines, and underwent PDC. Dental procedures included: oral hygiene guidance, restorative treatment of caries, surgical removal of residual roots and impacted or partially erupted teeth, cysts, supra and subgingival periodontal scaling and treatment of soft tissue disorders (removal of lesions and treatment of opportunistic infections). After 3 months, the clinical/laboratorial assessments were repeated. Results At inclusion, the Decayed, Missing and Filled Teeth (DMFT) index was higher in the pSS patients than in the control group (13.3 +/- 8.2 vs. 8.6 +/- 6.2, p=0.002), whereas periodontal parameters were comparable in both groups (p>0.05). After PDC, 26.9% of pSS patients showed a reduction of at least 6 points (clinical improvement) in XI, but mean XI remained unchanged (p=0.285). PDC resulted in an increase in mean unstimulated (p<0.001) and stimulated (p=0.001) salivary flow rates in pSS, with no change in salivary cytokine profile (p=0.05). Conclusion PDC promoted improvement in unstimulated and stimulated salivary flow rates in pSS. This novel finding reinforces the recommendation of this strategy for pSS patients. Clinicaltrials.gov (Identifier: NCT03711214).
  • article 2 Citação(ões) na Scopus
    A randomized controlled trial of an intervention promoting physical activity and healthy eating recommendations in systemic lupus erythematosus: the protocol study ""Living Well with Lupus""
    (2023) SIECZKOWSKA, Sofia Mendes; SMAIRA, Fabiana Infante; MAZZOLANI, Bruna Caruso; ROMERO, Marina; PASOTO, Sandra Gofinet; PINTO, Ana Lucia de Sa; LIMA, Fernanda Rodrigues; OLIVEIRA, Victor Rodrigues De; UEDA, Serli; BENATTI, Fabiana Braga; ROSCHEL, Hamilton; GUALANO, Bruno
    There is a paucity of studies assessing multidisciplinary interventions focused on tackling physical inactivity/sedentary behavior and poor dietary habits in SLE. The Living well with Lupus (LWWL) is a randomized controlled trial to investigate whether a six-month lifestyle change intervention will improve cardiometabolic risk factors (primary outcome) among systemic lupus erythematosus (SLE) patients with low disease activity (SLEDAI score & LE; 4) and with high cardiovascular risk. As secondary goals, we will evaluate: (1) the intervention's safety, efficacy, and feasibility in promoting lifestyle changes, and (2) the effects of the intervention on secondary outcomes (i.e., clinical parameters, functional capacity, fatigue, psychological aspects, sleep quality and health-related quality of life). Patients will be randomly allocated to either a control (i.e., standard care) or a lifestyle intervention group using a simple randomization (1:1 ratio, blocks of 20). Mixed Model analyses will be conducted for comparing groups following an intention-to-treat approach. A per protocol analysis will also be conducted. This study has the potential to generate new, clinically relevant data able to refine the multidisciplinary management of SLE patients. Protocol version number: NCT04431167 (first version).
  • article 28 Citação(ões) na Scopus
    Metabolic syndrome in Sjogren's syndrome patients: a relevant concern for clinical monitoring
    (2016) AUGUSTO, Kristopherson Lustosa; BONFA, Eloisa; PEREIRA, Rosa Maria Rodrigues; BUENO, Cleonice; LEON, Elaine Pires; VIANA, Vilma Santos Trindade; PASOTO, Sandra Gofinet
    Metabolic syndrome (MetS) has been described in autoimmune diseases. However, there are scarce data about MetS and adipocytokine profile in primary Sjogren's syndrome (pSS). Seventy-one female pSS patients (American-European Consensus Group Criteria, 2002) aged 18-65 years and 71 age-, race-matched control women were enrolled in this case-control study. Clinical data were collected by a standardized protocol. Blood levels of glucose, cholesterol, low-density lipoprotein-cholesterol (LDL-C), high-density lipoprotein-cholesterol (HDL-C), triglycerides, interleukin-1beta (IL-1beta)/IL-6, B-cell activating factor (BAFF), insulin, and leptin/adiponectin/visfatin/resistin were determined. Patients and controls were comparable regarding body mass index (BMI), smoking, sedentariness, and menopause (p > 0.05). MetS (39.4 vs. 16.9 %, p = 0.005), hypertension (p = 0.004), and dyslipidemia (p = 0.002) were more frequent in patients than controls. IL-1beta, IL-6, BAFF, resistin, and adiponectin levels were higher in patients than controls (p < 0.05). pSS patients with MetS (n = 28) had higher BMI, waist circumference, cholesterol, LDL-C, triglycerides, insulin, leptin and HOMA-IR values, and greater hypertension and diabetes rates than pSS patients without MetS (n = 43) (p < 0.05). Current and/or previous prednisone use (75.0 vs. 62.8 %, p = 0.313), current (3.0 +/- 4.5 vs. 1.6 +/- 3.2 mg/day, p = 0.299), and cumulative prednisone doses (p = 0.495) were similar in both groups. Otherwise, IL-1beta level was higher in MetS patients than in non-MetS patients (p = 0.012), and this finding was confirmed (p = 0.048) by multivariate analysis with adjustments for age, ethnicity, prednisone use, current and cumulative prednisone doses, and duration of use. We identified high MetS frequency and abnormal adipocytokine profile in pSS. The association of MetS with elevated IL-1beta level suggests that inflammation plays an important role in its pathogenesis.
  • article 6 Citação(ões) na Scopus
    Mortality risk factors in primary Sjogren syndrome: a real-world, retrospective, cohort study
    (2023) BRITO-ZERON, Pilar; FLORES-CHAVEZ, Alejandra; HORVATH, Ildiko Fanny; RASMUSSEN, Astrid; LI, Xiaomei; OLSSON, Peter; VISSINK, Arjan; PRIORI, Roberta; ARMAGAN, Berkan; HERNANDEZ-MOLINA, Gabriela; PRAPROTNIK, Sonja; QUARTUCCIO, Luca; INANC, Nevsun; OEZKIZILTAS, Burcuguel; BARTOLONI, Elena; SEBASTIAN, Agata; ROMAO, Vasco C.; SOLANS, Roser; PASOTO, Sandra G.; RISCHMUELLER, Maureen; GALISTEO, Carlos; SUZUKI, Yasunori; TREVISANI, Virginia Fernandes Moca; FUGMANN, Cecilia; GONZALEZ-GARCIA, Andres; CARUBBI, Francesco; JURCUT, Ciprian; SHIMIZU, Toshimasa; RETAMOZO, Soledad; ATZENI, Fabiola; HOFAUER, Benedikt; MELCHOR-DIAZ, Sheila; GHEITA, Tamer; LOPEZ-DUPLA, Miguel; FONSECA-AIZPURU, Eva; GIACOMELLI, Roberto; VAZQUEZ, Marcos; CONSANI, Sandra; AKASBI, Miriam; NAKAMURA, Hideki; SZANTO, Antonia; FARRIS, A. Darise; WANG, Li; MANDL, Thomas; GATTAMELATA, Angelica; KILIC, Levent; PIRKMAJER, Katja Perdan; ABACAR, Kerem; TUFAN, Abdurrahman; VITA, Salvatore de; BOOTSMA, Hendrika; RAMOS-CASALS, Manuel
    Background What baseline predictors would be involved in mortality in people with primary Sjogren syndrome (SjS) remains uncertain. This study aimed to investigate the baseline characteristics collected at the time of diagnosis of SjS associated with mortality and to identify mortality risk factors for all-cause death and deaths related to systemic SjS activity measured by the ESSDAI score.Methods In this international, real-world, retrospective, cohort study, we retrospectively collected data from 27 countries on mortality and causes of death from the Big Data Sjogren Registry. Inclusion criteria consisted of fulfilling 2002/2016 SjS classification criteria, and exclusion criteria included chronic HCV/HIV infections and associated systemic autoimmune diseases. A statistical approach based on a directed acyclic graph was used, with all-cause and Sjogren-related mortality as primary endpoints. The key determinants that defined the disease phenotype at diagnosis (glandular, systemic, and immunological) were analysed as independent variables.Findings Between January 1st, 2014 and December 31, 2023, data from 11,372 patients with primary SjS (93.5% women, 78.4% classified as White, mean age at diagnosis of 51.1 years) included in the Registry were analysed. 876 ( 7.7%) deaths were recorded after a mean follow-up of 8.6 years (SD 7.12). Univariate analysis of prognostic factors for all-cause death identified eight Sjogren-related variables (ocular and oral tests, salivary biopsy, ESSDAI, ANA, anti-Ro, anti-La, and cryoglobulins). The multivariate CPH model adjusted for these variables and the epidemiological features showed that DAS-ESSDAI (high vs no high: HR = 1.68; 95% CI, 1.27-2.22) and cryoglobulins (positive vs negative: HR = 1.72; 95% CI, 1.22-2.42) were independent predictors of all cause death. Of the 640 deaths with available information detailing the specific cause of death, 14% were due to systemic SjS. Univariate analysis of prognostic factors for Sjogren-cause death identified five Sjogrenrelated variables (oral tests, clinESSDAI, DAS-ESSDAI, ANA, and cryoglobulins). The multivariate competing risks CPH model adjusted for these variables and the epidemiological features showed that oral tests (abnormal vs normal results: HR = 1.38; 95% CI, 1.01-1.87 ), DAS-ESSDAI (high vs no high: HR = 1.55; 95% CI, 1.22-1.96 ) and cryoglobulins (positive vs negative: HR = 1.52; 95% CI, 1.16-2) were independent predictors of SjS-related death.Interpretation The key mortality risk factors at the time of SjS diagnosis were positive cryoglobulins and a high systemic activity scored using the ESSDAI, conferring a 2-times increased risk of all-cause and SjS-related death. ESSDAI measurement and cryoglobulin testing should be considered mandatory when an individual is diagnosed with SjS.Funding Novartis.Copyright & COPY; 2023 The Author(s).