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

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:

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

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Corrigendum

Acknowledgment

The authors wish to thank David Buss for his editorial assistance.

Financial & competing interests disclosure

This work was supported by Grants La Marató de TV3 (071810), Fondo de Investigaciones Sanitarias (1201009), and ‘Ajut per a la Recerca Josep Font’, Hospital Clinic-Barcelona (PBZ, 2012). The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Key issues

• Oral and ocular dryness are the principal symptoms leading to a suspicion of SjS.

• Episodic parotid gland swelling is a frequent presentation of the disease, especially in children.

• Systemic features may develop either at the time of diagnosis of SjS or later during the follow-up.

• In patients with systemic involvement, other processes unrelated to SjS should always be ruled out.

• Coexisting laboratory abnormalities may support a clinical suspicion of an occult SjS.

• Focal lymphocytic sialadenitis and anti-Ro/SS-A, La/SS-B antibodies should be searched for in patients with a suspected occult SjS.

• Systemic patients without anti-Ro/SS-A antibodies should be biopsied to confirm occult disease.

• A positive immunological/histopathological result will lead to an early diagnosis of SjS several years before the onset of overt sicca syndrome.

• An early, accurate diagnosis of SjS may prevent, or ensure timely treatment of, SjS-related complications.

• A collaborative multidisciplinary diagnostic approach is essential in systemic patients.