EULAR Primary SjöGren’s Syndrome Disease Activity Index (ESSDAI), Results from the National Registry of the Spanish Society of Rheumatology (SJÖGRENSER)

José Rosas¹, Carlos Sánchez-Piedra², Monica Fernandez Castro³, Jose Luis Andreu⁴, Víctor Martínez Taboada⁵, Alejandro Olivé^6,7, Enrique Judez⁸, Clara Moriano⁹, Vicente Torrente-Segarra¹⁰, Hector Corominas¹¹, Blanca García Magallon¹², Cristina Bohórquez Heras¹³, Javier Loricera¹⁴, Joaquin Maria Belzunegui Otano¹⁵, Carlos Guillén-Astete¹⁶, Ivan Castellvi¹⁷, José Miguel Senabre-Gallego¹, Ana Pons¹ and on behalf of SJOGRENSER project (GEEAS-SER), ¹Rheumatology, Hospital Marina Baixa, Villajoyosa (Alicante), Spain, ²Research Unit, Spanish Society of Rheumatology, Madrid, Spain, ³Rheumatology, Hospital Infanta Sofía, Madrid, Spain, ⁴Rheumatology, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain, ⁵Rheumatology, Hospital Marqués de Valdecilla, Santander, Spain, ⁶Rheumatology, Hospital Universitario Germans Trias i Pujol, Barcelona, Spain, ⁷Rheumatology, Hospital Germans Trias i Pujol, Badalona, Spain, ⁸Rheumatology Department. Hospital de Albacete, Albacete, Spain, ⁹Rheumatology, Hospital de León, Leon, Spain, ¹⁰Hospital de L'Hospitalet, L'Hospitalet de Llobregat, Spain, ¹¹Hospital Sant Joan Despí Moisès Broggi, Barcelona, Spain, ¹²Rheumatology, Hospital Miguel Servet, Zaragoza, Spain, ¹³University Hospital Príncipe de Asturias, Immune System Diseases, Rheumatology department, Alcalá de Henares, Madrid, Spain, ¹⁴Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Santander, Spain, ¹⁵Rheumatology, Donostia University Hospital, Donostia, Spain, ¹⁶Rheumatology, Hospital Ramón y Cajal, Madrid, Spain, ¹⁷Rheumatology, Hospital Santa Creu i San Pau, Barcelona, Spain

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: Sjogren's syndrome and registry

Session Information

Date: Tuesday, November 15, 2016

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

Session Type: ACR Poster Session C

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

Background/Purpose: Primary Sjögren’s syndrome (PSS) is a systemic disease primarily characterized by lymphocytic infiltration of exocrine glands, but lymphocytic infiltration, can also can affect extraglandular tissues. Recently, it has been validated the EULAR SS disease activity index (ESSDAI), that is a clinical index that measure disease activity in PSS. The ESSDAI, includes 12 domains (organ systems: cutaneous, respiratory, renal, articular, muscular, peripheral nervous system, central nervous system, haematological, glandular, constitutional, lymphadenopathic, biological). The ESSDAI is now in use as a gold standard to measure disease activity in clinical studies, and as an outcome measure. The purpose of this study is to evaluate the ESSDAI baseline characteristics in a large Spanish multicenter registry of PSS.

Methods: SJÖGRENSER, is a multicenter descriptive transversal study of a cohort of PSS patients fulfilling European/American consensus criteria 2012, collected from Rheumatology clinics all over Spain. Patients were included by randomisation from an anonymised list provided by every department. Two hundred and ninety eight variables were investigated: epidemiological, clinical, serological characteristics, treatments and complications. Specifically, ESSDAI domains were collected. The score may vary between 0-123. It is considered low activity an ESSDAI <5; moderate activity 5-13, and high activity if ESSDAI is ≥14. Variables were analysed by descriptive statistical methods, using means, medians, and rates, with their deviations and interquartile ranges (p25-p75).

Results: Of 437 patients included, 95% were women, with median age (p25-75): 58.63 years (50,02-67,98 years), and age at diagnosis of 50.24 years (42,99-58,29 years), with a disease evolution of disease of 10.4 years (6-16 years). The median of diagnosis criteria of SS was 5 (4-5). Minor salivary gland biopsy was positive in 69%. Rheumatoid factor, AAN, anti-Ro/SSA and anti-La/SSB and cryoglobulins, were positive in 64,76%, 62,23 %, 93,59%, 67,05%, 3% of patients respectively. Cytopenia was present in 27% of patients and Beta-2 microglobulin serum levels was increased in 22% and C3/C4 reduced in 23% of patients. The median of ESSDAI of our population at enter to the study was 2 (0-4). In 31% of patients, ESSDAI, was 0, in 49% was concordant with low activity, moderate activity in 15% and 5% of patients show high activity. In table, the domains of ESSDAI, at enter to SJÖGREN-SER are shown. Table. ESSDAI results at enter at SJÖGRENSER cohort.

ESSDAI domains	SJÖGRENSER N: 437 %
Constitutional	8
Lymphadenopathy	2
Glandular	4
Articular	35
Cutaneous	3
Pulmonary	6
Renal	5
Muscular	0,2
Peripheral nevous system	3
Central nervous system	2
Haematological	27
Biological	28

Conclusion: 1. In SJÖGRENSER, 20% of patients show moderate-high ESSDAI results and haematological, biological and articular domains are present in more than 10% of patients. 2. ESSDAI is a suitable tool to define and compare cohorts of patients with PSS.

Disclosure: J. Rosas, None; C. Sánchez-Piedra, None; M. Fernandez Castro, None; J. L. Andreu, None; V. Martínez Taboada, None; A. Olivé, None; E. Judez, None; C. Moriano, None; V. Torrente-Segarra, None; H. Corominas, None; B. García Magallon, None; C. Bohórquez Heras, None; J. Loricera, None; J. M. Belzunegui Otano, None; C. Guillén-Astete, None; I. Castellvi, None; J. M. Senabre-Gallego, None; A. Pons, None.

To cite this abstract in AMA style:

Rosas J, Sánchez-Piedra C, Fernandez Castro M, Andreu JL, Martínez Taboada V, Olivé A, Judez E, Moriano C, Torrente-Segarra V, Corominas H, García Magallon B, Bohórquez Heras C, Loricera J, Belzunegui Otano JM, Guillén-Astete C, Castellvi I, Senabre-Gallego JM, Pons A. EULAR Primary SjöGren’s Syndrome Disease Activity Index (ESSDAI), Results from the National Registry of the Spanish Society of Rheumatology (SJÖGRENSER) [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/eular-primary-sjogrens-syndrome-disease-activity-index-essdai-results-from-the-national-registry-of-the-spanish-society-of-rheumatology-sjogrenser/. Accessed January 20, 2021.

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