Severe, life-threatening phenotype of primary Sjögren's syndrome: clinical characterisation and outcomes in 1580 patients (GEAS-SS Registry)

To analyse the clinical features and outcomes of patients presenting with life-threatening systemic disease in a large cohort of Spanish patients with primary Sjögren's syndrome (SS). The GEAS-SS multicentre registry was formed in 2005 with the aim of collecting a large series of Spanish patien...

Full description

Saved in:
Bibliographic Details
Published in:Clinical and experimental rheumatology 2018-05, Vol.36 Suppl 112 (3), p.121-129
Main Authors: Flores-Chávez, Alejandra, Kostov, Belchin, Solans, Roser, Fraile, Guadalupe, Maure, Brenda, Feijoo-Massó, Carlos, Rascón, Francisco-Javier, Pérez-Alvarez, Roberto, Zamora-Pasadas, Mónica, García-Pérez, Alicia, Lopez-Dupla, Miguel, Duarte-Millán, Miguel-Ángel, Ripoll, Mar, Fonseca-Aizpuru, Eva, Guisado-Vasco, Pablo, Pinilla, Blanca, de-la-Red, Gloria, Chamorro, Antonio-J, Morcillo, César, Fanlo, Patricia, Soto-Cárdenas, Mª José, Retamozo, Soledad, Ramos-Casals, Manuel, Brito-Zerón, Pilar
Format: Article
Language:English
Subjects:
Adult
Aged
Decision Support Techniques
Disease Progression
Female
Glucocorticoids - therapeutic use
Humans
Immunosuppressive Agents - therapeutic use
Male
Middle Aged
Phenotype
Predictive Value of Tests
Registries
Risk Assessment
Risk Factors
Severity of Illness Index
Sjogren's Syndrome - diagnosis
Sjogren's Syndrome - epidemiology
Sjogren's Syndrome - mortality
Sjogren's Syndrome - therapy
Spain - epidemiology
Treatment Outcome
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:To analyse the clinical features and outcomes of patients presenting with life-threatening systemic disease in a large cohort of Spanish patients with primary Sjögren's syndrome (SS). The GEAS-SS multicentre registry was formed in 2005 with the aim of collecting a large series of Spanish patients with primary SS, and included more than 20 Spanish reference centres with substantial experience in the management of SS patients. By January 2018, the database included 1580 consecutive patients fulfilling the 2002 classification criteria for primary SS. Severe, life-threatening systemic disease was defined as an activity level scored as "high" in at least one ESSDAI domain. Among 1580 patients, 208 (13%) were classified as presenting a severe, potentially life-threatening systemic disease: 193 presented one ESSDAI domain classified as high, 14 presented two high scored domains and only one presented three high activity domains. The ESSDAI domains involved consisted of lymphadenopathy in 78 (37%) cases, CNS in 28 (13%), PNS in 25 (12%), pulmonary in 25 (12%), renal in 21 (10%), cutaneous in 19 (9%), articular in 18 (9%), haematological in 7 (3%) and muscular in 4 (2%). Patients with severe systemic disease were more frequently men (p=0.001) and had a higher frequency of anaemia (p
ISSN:0392-856X
1593-098X