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

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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

Retamozo, S. (författare): UNC, Consejo Nacl Invest Cient & Tecn CONICET, Inst Invest Ciencias Salud INICSA, Cordoba, Argentina;IUCBC, Cordoba, Argentina

Acar-Denizli, N. (författare): Mimar Sinant Fine Arts Univ, Fac Sci & Letters, Dept Stat, Istanbul, Turkey

Rasmussen, A. (författare): Oklahoma Med Res Fdn, Arthrit & Clin Immunol Res Programme, 825 NE 13th St, Oklahoma City, OK 73104 USA

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Horvath, I. F. (författare): Univ Debrecen, Fac Med, Div Clin Immunol, Debrecen, Hungary

Baldini, C. (författare): Univ Pisa, Rheumatol Unit, Pisa, Italy

Priori, R. (författare): Sapienza Univ Rome, Rheumatol Clin, Dept Internal Med & Med Specialties, Rome, Italy

Sandhya, P. (författare): Christian Med Coll & Hosp, Dept Clin Immunol & Rheumatol, Vellore, Tamil Nadu, India

Hernandez-Molina, G. (författare): Inst Nacl Ciencias Med & Nutr Salvador Zubiran, Immunol & Rheumatol Dept, Mexico City, DF, Mexico

Armagan, B. (författare): Hacettepe Univ, Fac Med, Dept Internal Med, Ankara, Turkey

Praprotnik, S. (författare): Univ Med Ctr, Dept Rheumatol, Ljubljana, Slovenia

Kvarnstrom, M. (författare): Karolinska Institutet

Gerli, R. (författare): Univ Perugia, Dept Med, Rheumatol Unit, Perugia, Italy

Sebastian, A. (författare): Wroclaw Med Univ, Dept Rheumatol & Internal Med, Wroclaw, Poland

Solans, R. (författare): Hosp Valle De Hebron, Dept Internal Med, Barcelona, Spain

Rischmueller, M. (författare): Queen Elizabeth Hosp, Dept Rheumatol, Adelaide, SA, Australia;Univ Adelaide, Adelaide, SA, Australia

Pasoto, S. G. (författare): Univ Sao Paulo, Fac Med, HCFMUSP, Hosp Clin,Rheumatol Div, Sao Paulo, Brazil

Valim, V. (författare): Univ Fed Espirito Santo, Dept Med, Vitoria, ES, Brazil

Nordmark, Gunnel (författare): Uppsala universitet,Reumatologi

Kruize, A. A. (författare): Univ Med Ctr Utrecht, Dept Rheumatol & Clin Immunol, Utrecht, Netherlands

Nakamura, H. (författare): Nagasaki Univ, Grad Sch Biomed Sci, Div Adv Prevent Med Sci, Dept Immunol & Rheumatol, Nagasaka, Yamanashi, Japan

Hofauer, B. (författare): Univ Freiburg, Otorhinolaryngol Head & Neck Surg, Freiburg, Germany

Giacomelli, R. (författare): Univ Aquila, Sch Med, Clin Unit Rheumatol, Laquila, Italy

Fernandes Moca Trevisani, V. (författare): Univ Fed Sao Paulo, Sao Paulo, Brazil

Devauchelle-Pensec, V. (författare): Brest Univ Hosp, Rheumatol Dept, Paris, France

Atzeni, F. (författare): Univ Messina, Inst Milan, IRCCS Galeazzi Orthopaed, Messina, Italy;Univ Messina, Rheumatol Unit, Messina, Italy

Gheita, T. A. (författare): Cairo Univ, Kasr Al Amny Sch Med, Rheumatol Dept, Cairo, Egypt

Consani-Fernandez, S. (författare): Hosp Maciel, Internal Med, Montevideo, Uruguay;Univ Republ UdelaR, Montevideo, Uruguay

Szanto, A. (författare): Univ Debrecen, Fac Med, Div Clin Immunol, Debrecen, Hungary

Sivils, K. (författare): Oklahoma Med Res Fdn, Arthrit & Clin Immunol Res Programme, 825 NE 13th St, Oklahoma City, OK 73104 USA

Gattamelata, A. (författare): Sapienza Univ Rome, Rheumatol Clin, Dept Internal Med & Med Specialties, Rome, Italy

Danda, D. (författare): Christian Med Coll & Hosp, Dept Clin Immunol & Rheumatol, Vellore, Tamil Nadu, India

Kilic, L. (författare): Hacettepe Univ, Fac Med, Dept Internal Med, Ankara, Turkey

Bartoloni, E. (författare)

Bombardieri, S. (författare)

Sanchez-Guerrero, J. (författare): Inst Nacl Ciencias Med & Nutr Salvador Zubiran, Immunol & Rheumatol Dept, Mexico City, DF, Mexico

Wahren-Herlenius, M. (författare): Karolinska Institutet

Mariette, X. (författare): Univ Paris Sud, Ctr Immunol Viral Infect & Autoimmune Dis, Hop Univ Paris Sud, AP HP,UMR1184,INSERM, Paris, France

Ramos-Casals, M. (författare): Univ Barcelona, Sjogrens Syndrome Res Grp AGAUR, Lab Autoimmune Dis Josep Font,Hosp Clin, IDIBAPS CELLEX,Dept Autoimmune Dis ICMiD, Barcelona, Spain

Brito-Zeron, P. (författare): Univ Barcelona, Sjogrens Syndrome Res Grp AGAUR, Lab Autoimmune Dis Josep Font,Hosp Clin, IDIBAPS CELLEX,Dept Autoimmune Dis ICMiD, Barcelona, Spain;Hosp CIMA Sanitas, Dept Med, Autoinmmne Dis Unit, Barcelona, Spain

Nakamura, T (författare)

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CLINICAL & EXPER RHEUMATOLOGY, 2019
2019
Engelska.
Ingår i: Clinical and Experimental Rheumatology. - : CLINICAL & EXPER RHEUMATOLOGY. - 0392-856X .- 1593-098X. ; 71

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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.

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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

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