Isolated Anti-La/SS-B Positivity in Patients Diagnosed with Primary Sjogren Syndrome: Analysis of 222 Patients from the Sjogren Big Data Cohort

Pilar Brito-Zerón^1,2,3, Nihan Acar-Denizli⁴, Margit Zeher⁵, Astrid Rasmussen⁶, Raphaele Seror⁷, Elke Theander⁸, Xiaomei Li⁹, Chiara Baldini¹⁰, Jacques-Eric Gottenberg¹¹, Debashish Danda¹², Luca Quartuccio¹³, Roberta Priori^14,15, Gabriela Hernandez-Molina¹⁶, Aike A. Kruize¹⁷, Valeria Valim¹⁸, Marika Kvarnström¹⁹, Damien Sene²⁰, Roberto Gerli²¹, Sonja Praprotnik²², David A. Isenberg²³, Roser Solans²⁴, Maureen Rischmueller²⁵, Seung-Ki Kwok²⁶, Gunnel Nordmark²⁷, Yasunori Suzuki²⁸, Roberto Giacomelli²⁹, Valerie Devauchelle-Pensec³⁰, Michele Bombardieri³¹, Benedikt Hofauer³², Hendrika Bootsma³³, Johan G. Brun³⁴, Guadalupe Fraile³⁵, Steven E. Carsons³⁶, Tamer Gheita³⁷, Jacques Morel³⁸, Cristina F. Vollenweider³⁹, Fabiola Atzeni⁴⁰, Soledad Retamozo⁴¹, Ildike-Fanny Horvath⁵, Kathy Sivils⁶, Thomas Mandl⁴², Pulukool Sandhya¹², Salvatore De Vita⁴³, Jorge Sánchez-Guerrero⁴⁴, Eefje van der Heijden¹⁷, Virginia Moça Trevisano⁴⁵, Marie Wahren-Herlenius¹⁹, Manuel Ramos-Casals^3,46,47 and EULAR-SS Task Force Big Bata Consortium (ASSES, GEAS-SEMI EULAR), ¹Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA- Sanitas, Barcelona., Bacelona, Spain, ²Laboratory of Systemic Autoimmune Diseases “Josep Font”, CELLEX, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Systemic Autoimmune Diseases, ICMID, Hospital Clinic, Barcelona, Barcelona, Spain, ³Laboratory of Systemic Autoimmune Diseases “Josep Font”, CELLEX, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Systemic Autoimmune Diseases, ICMID, Hospital Clinic, Barcelona, Spain, Barcelona, Spain, ⁴Department of Statistics, Faculty of Science and Letters, Mimar Sinan Fine Arts University, Istambul, Turkey, ⁵Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Debrecen, Hungary., Debrecen, Hungary, ⁶Arthritis and Clinical Immunology Research Program, Oklahoma Medical Research Foundation, USA, Oklahoma City, OK, ⁷Center for Immunology of Viral Infections and Autoimmune Diseases, Assistance Publique – Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Le Kremlin-Bicêtre, Université Paris Sud, INSERM, Paris, France, Paris, France, ⁸Department of Rheumatology, Malmö University Hospital, Lund University, Sweden, Malmö, Sweden, ⁹Department of Rheumatology and Immunology, Anhui Medical University Affiliated Provincial Hospital, China, Hefei, Anhui, China, ¹⁰University of Pisa, Rheumatology Unit, University of Pisa, Italy, Pisa, Italy, ¹¹Department of Rheumatology, Strasbourg University Hospital, Université de Strasbourg, CNRS, Strasbourg, France, Strasbourg, France, ¹²Clinical Immunology & Rheumatology, Christian Medical College, Vellore, India, Vellore, India, ¹³Rheumatology Clinic, DSMB, University of Udine, Udine, Italy, Udine, Italy, ¹⁴Rheumatology Unit, Sapienza University of Rome, Rome, Italy, ¹⁵Department of Internal Medicine and Medical Specialties, Sapienza University, Rome, Italy, ¹⁶Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico City, Mexico, ¹⁷Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands, Utrecht, Netherlands, ¹⁸Department of Medicine, Universidade Federal do Espírito Santo, Vitória, Brazil, Vitória, Brazil, ¹⁹Department of Medicine, Solna, Unit of Experimental Rheumatology, Karolinska Institutet, and Karolinska University Hospital, Stockholm, Sweden, Stockholm, Sweden, ²⁰Service de Médecine Interne 2, Hôpital Lariboisière, Université Paris VII, Assistance Publique-Hôpitaux de Paris, 2, Paris, France, Paris, France, ²¹Clinical and Experimental Medicine, Rheumatology Unit, University of Perugia, Italy, Perugia, Italy, ²²Department of Rheumatology, University Medical Centre Ljubljana, Slovenia, Ljubljana, Slovenia, ²³Centre for Rheumatology Research, University College Hospital London, UK, London, United Kingdom, ²⁴Autoimmune Systemic Diseases Unit, Department of Internal Medicine, Hospital Vall d'Hebron, Autonomous University of Barcelona, Spain, Barcelona, Spain, ²⁵Department of Rheumatology, School of Medicine, The University of Western Australia, Crawley, Australia, Crawley, Australia, ²⁶Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, The Catholic University of Korea College of Medicine, Seoul, South Korea, ²⁷Rheumatology, Department of Medical Sciences, Uppsala University, Sweden, Uppsala, Sweden, ²⁸Division of Rheumatology , Kanazawa University Hospital , Kanazawa , Ishikawa , Japan, Ishikawa, Japan, ²⁹Clinical Unit of Rheumatology, University of l'Aquila, School of Medicine, L'Aquila, Italy, L'Aquila, Italy, ³⁰Department of Rheumatology, Brest University Hospital, Brest, France, Brest, France, ³¹Willian Harvey Research Institute, Centre for Experimental Medicine and Rheumatology, QMUL, UK, London, United Kingdom, ³²Hals-Nasen-Ohrenklinik und Poliklinik, Technische Universität München, München, Germany, München, Germany, ³³Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, The Netherlands, Groningen, Netherlands, ³⁴Department of Clinical Science, University of Bergen. Department of Rheumatology, Haukeland University Hospital, Bergen, Norway, Bergen, Norway, ³⁵Department of Internal Medicine, Hospital Ramón y Cajal, Madrid, Spain, Madrid, Spain, ³⁶Division of Rheumatology, Allergy and Immunology Winthrop-University Hospital, Stony Brook University School of Medicine, NY, USA, Mineola, NY, ³⁷Rheumatology, Rheumatology Department, Faculty of Medicine, Cairo University, Egypt, Cairo, Egypt, ³⁸Department of Rheumatology, Teaching hospital and University of Montpellier, France, Montpellier, France, ³⁹Rheumatology, German Hospital, Buenos Aires, Argentina, Buenos Aires, Argentina, ⁴⁰IRCCS Galeazzi Orthopedic Institute, Milan, Italy, Milan, Italy, ⁴¹Rheumatology Unit, Hospital Privado Centro Médico de Córdoba, Argentina, Córdoba, Argentina, ⁴²Department of Rheumatology, Malmö University Hospital, Lund University, Sweden, Lund, Sweden, ⁴³Clinic of Rheumatology, Department of Medical and Biological Sciences, University Hospital "Santa Maria della Misericordia", Udine, Italy, Udine, Italy, ⁴⁴Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, Mexico, ⁴⁵Federal University of São Paulo, Sao Paulo, Brazil, San Paulo, Brazil, ⁴⁶Sjögren Syndrome Research Group (AGAUR), Barcelona, Spain, ⁴⁷Department of Medicine, University of Barcelona, Barcelona, Spain., Barcelona, Spain

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: antibodies and big data, Sjogren's syndrome

Session Information

Date: Tuesday, November 15, 2016

Title: Sjögren's Syndrome - Poster II: Clinical Science

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: To describe the clinical, immunological and histopathological profile of patients with primary Sjögren syndrome (SjS) presenting with isolated circulating anti-La/SS-B antibodies in the absence of concomitant anti-Ro/SS-B antibodies in a large international cohort of patients.

Methods: The Big Data Sjögren Project Consortium is an international, multicentre registry of primary SjS patients fulfilling the 2002 AE classification criteria. By January 2016, the participant centres had included 8310 patients. Determination of anti-Ro/SS-A and anti-SS-B antibodies were made using commercial tests used in daily practice. Patients were classified in 4 groups: A) Double positivity (Ro+La+); B) Isolated Ro positivity (Ro+La-); C) Isolated La positivity (Ro-La+); and D) Biopsy-proven immunonegative (Ro-La-).

Results: Anti-Ro/SS-A and anti-SS-B antibodies were tested in 8213 (99%) patients: 3372 (41%) were Ro+La+, 2546 (31%) Ro+La-, 222 (3%) Ro-La+ and 2073 (25%) were biopsy-proven Ro-La-. Epidemiologically, age at diagnosis of Ro-La+ patients was similar to the two Ro+ groups and was 5.5 years lower than that observed in Ro-La- patients (p<0.001). Clinically, Ro-La+ patients had a similar frequency of ocular dryness than Ro-La- patients (a figure significantly higher in comparison with the two Ro+ groups, p<0.001), while the frequency of oral dryness was similar to the Ro+ groups and significantly lower than that reported for the Ro-La- group (p<0.001) (Figure 1). With respect to diagnostic tests, Ro-La+ patients showed similar figures in comparison with the other groups but a significant higher frequency in abnormal diagnostic tests (salivary flows and parotid scintigraphy, p<0.001). Minor salivary gland biopsy was carried out in 147 (66%) out of the 222 Ro-La+ patients, and showed Chisholm-Mason 3-4 grades in 103/147 (70%), a figure significantly lower than that reported for the 2 Ro+ groups (p<0.001). Immunologically, Ro-La+ patients showed a similar immunological profile than that reported for Ro-La- patients, with a significant lower frequency of ANA, RF, hypocomplementemia and cryoglobulinemia in comparison with the 2 Ro+ groups (p<0.001, Figure 1).

Conclusion: In the largest reported cohort of patients with primary SjS fulfilling the 2002 AE classification criteria, only 3% of patients had isolated anti-La/SS-B antibodies. This small subset of patients presents a specific clinical and immunological profile that mix some features characteristic of immunonegative patients and some of patients carrying anti-Ro/SS-A antibodies. Further studies are required to confirm this isolated anti-La positivity and should evaluate the differences in systemic activity and outcomes of this subset of patients with respect to the other immunological groups.

Disclosure: P. Brito-Zerón, None; N. Acar-Denizli, None; M. Zeher, None; A. Rasmussen, None; R. Seror, None; E. Theander, None; X. Li, None; C. Baldini, None; J. E. Gottenberg, None; D. Danda, None; L. Quartuccio, None; R. Priori, None; G. Hernandez-Molina, None; A. A. Kruize, None; V. Valim, None; M. Kvarnström, None; D. Sene, None; R. Gerli, None; S. Praprotnik, None; D. A. Isenberg, None; R. Solans, None; M. Rischmueller, None; S. K. Kwok, None; G. Nordmark, None; Y. Suzuki, None; R. Giacomelli, None; V. Devauchelle-Pensec, None; M. Bombardieri, None; B. Hofauer, None; H. Bootsma, None; J. G. Brun, None; G. Fraile, None; S. E. Carsons, None; T. Gheita, None; J. Morel, None; C. F. Vollenweider, None; F. Atzeni, None; S. Retamozo, None; I. F. Horvath, None; K. Sivils, None; T. Mandl, None; P. Sandhya, None; S. De Vita, None; J. Sánchez-Guerrero, None; E. van der Heijden, None; V. Moça Trevisano, None; M. Wahren-Herlenius, None; M. Ramos-Casals, None.

To cite this abstract in AMA style:

Brito-Zerón P, Acar-Denizli N, Zeher M, Rasmussen A, Seror R, Theander E, Li X, Baldini C, Gottenberg JE, Danda D, Quartuccio L, Priori R, Hernandez-Molina G, Kruize AA, Valim V, Kvarnström M, Sene D, Gerli R, Praprotnik S, Isenberg DA, Solans R, Rischmueller M, Kwok SK, Nordmark G, Suzuki Y, Giacomelli R, Devauchelle-Pensec V, Bombardieri M, Hofauer B, Bootsma H, Brun JG, Fraile G, Carsons SE, Gheita T, Morel J, Vollenweider CF, Atzeni F, Retamozo S, Horvath IF, Sivils K, Mandl T, Sandhya P, De Vita S, Sánchez-Guerrero J, van der Heijden E, Moça Trevisano V, Wahren-Herlenius M, Ramos-Casals M. Isolated Anti-La/SS-B Positivity in Patients Diagnosed with Primary Sjogren Syndrome: Analysis of 222 Patients from the Sjogren Big Data Cohort [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/isolated-anti-lass-b-positivity-in-patients-diagnosed-with-primary-sjogren-syndrome-analysis-of-222-patients-from-the-sjogren-big-data-cohort/. Accessed .

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