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Expert Rev Clin Immunol. 2016;12(2):137-56. doi: 10.1586/1744666X.2016.1109449. Epub 2015 Dec 22.

Early diagnosis of primary Sjögren's syndrome: EULAR-SS task force clinical recommendations.

Author information

1
a Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, CELLEX-IDIBAPS, Department of Autoimmune Diseases, ICMiD , Hospital Clínic , Barcelona , Spain.
2
b Department of Rheumatology , Skane University Hospital Malmö, Lund University , Sweden.
3
c Rheumatology Unit , University of Pisa , Pisa , Italy.
4
d Department of Rheumatology , Assistance Publique-Hopitaux de Paris, Hôpital Bicêtre, Le Kremlin Bicêtre , Paris , France.
5
e Department of Rheumatology , Université Paris-Sud, Le Kremlin Bicêtre , Paris , France.
6
f Hospital Privado Centro Médico , Córdoba , Argentina.
7
g Clinic of Rheumatology, Department of Medical and Biological Sciences , University Hospital Santa Maria della Misericordia , Udine , Italy.
8
h Department of Rheumatology and Clinical Immunology , University Medical Center Groningen, University of Groningen , Groningen , the Netherlands.
9
i Rheumatology Department , University Hospitals Birmingham NHS Foundation Trust , Birmingham , UK.
10
j Rheumatology Department , Charité, University Hospital , Berlin , Germany.
11
k Department of Rheumatology , Strasbourg University Hospital, Université de Strasbourg , Strasbourg , France.
12
l Musculoskeletal Research Group, Institute of Cellular Medicine , Newcastle University , Newcastle upon Tyne , UK.
13
m Department of Rheumatology and Clinical Immunology , University Medical Center Utrecht , Utrecht , the Netherlands.
14
n Department of Pathophysiology , School of Medicine, University of Athens , Greece.
15
o Istituto San Giuseppe , Como and Casa di Cura Lecco , Lecco , Italy.
16
p Division of Rheumatology, Department of Medicine , New York University School of Medicine , New York , NY , USA.
17
q Department of Rheumatology , Scripps Memorial Hospital-XiMED , La Jolla , CA , USA.

Erratum in

Abstract

Sjögren's syndrome (SjS) is a systemic autoimmune disease that mainly affects the exocrine glands, leading to generalized mucosal dryness. However, primary SjS may initially present with non-sicca (systemic) manifestations. When these features appear before the onset of an overt sicca syndrome, we may talk of an underlying 'occult' SjS. The European League Against Rheumatism (EULAR) has promoted and supported an international collaborative study group (EULAR-SS Task Force) aimed at developing consensual recommendations to provide a homogeneous approach to the patient with primary SjS presenting with systemic involvement. This review summarizes the key factors that should be taken into account in the diagnostic approach in a patient with suspected SjS according to the main clinical patterns of presentation, and is especially focused on organ-specific systemic disease presentations, including a consensus set of recommendations in order to reach an early diagnosis. Close collaboration with the different specialties involved through a comprehensive multidisciplinary approach is essential in SjS patients presenting with systemic involvements.

KEYWORDS:

ESSDAI; Sjögren syndrome; anti-Ro/SS-A antibodies; classification criteria; early diagnosis; focal lymphocytic sialadenitis; systemic activity

PMID:
26691952
DOI:
10.1586/1744666X.2016.1109449
[Indexed for MEDLINE]

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