Sicca syndrome (dry mouth or eyes): suspected Sjögren's disease
BioMG follows French guidelines. Adapt it to the guidelines and the tests available where you practise.
Disabling dry mouth or dry eyes: suspected Sjögren's disease
Laboratory tests (state "suspected Sjögren syndrome" on the request form)
- Antinuclear antibodies (on HEp-2 cells transfected with Ro60/SSA) with anti-SSA/Ro60 and anti-SSB/La antibodies
- Rheumatoid factor
- Serum protein electrophoresis
- FBCif neededPart of the initial laboratory tests of the PNDS (cytopenias, lymphopenia) [2].
- TSH (if signs of thyroid dysfunction)if neededThyroid dysfunction is among the causes of dryness other than Sjögren's disease [2].
- Hepatitis C serology (if risk factor)if neededHepatitis C is a cause of dryness to be ruled out [2].
- HIV serology (if swelling of the parotid or submandibular glands)if neededHIV is among the general causes of parotid enlargement to look for, like sarcoidosis, IgG4-related disease or MALT lymphoma [2].
- HbA1c (if known diabetes, to assess control)if neededPoorly controlled diabetes is a cause of dryness [2].
Also consider
- Anti-Ro60 positive? Anti-Ro60 antibodies also point towards lupus: see the page "Lupus systémique".
Do not order
- Follow-up anti-SSA or anti-SSB antibodies: they remain positive for life and their titre does not track activity; do not repeat [2].
Confirmed Sjögren's disease: laboratory monitoring
At every follow-up visit (every 3 to 6 months if active disease, every 1 to 2 years if quiescent)
- FBC
- Serum electrolytes
- Serum creatinine with eGFR
- CRP
- Serum albumin
- AST
- ALT
- GGT
- Bilirubin
- Alkaline phosphatase
- Creatine kinase (CK)
- Serum protein electrophoresis
- IgG
- Urine protein:creatinine ratio (spot sample)
- Rheumatoid factor, complement C3 and C4, cryoglobulins (if cryoglobulin positive at baseline, vasculitis, renal involvement or progressive disease)if neededThe PNDS reserves them for patients with initial cryoglobulinaemia or signs of active disease, vasculitis or glomerular renal involvement [2].
- LDH (if lymphoma suspected)if neededNot a routine test, if lymphoma is suspected [2].
- Hepatitis B serologyif neededBefore starting an immunosuppressive treatment [2].
- Hepatitis C serologyif neededBefore starting an immunosuppressive treatment [2].
- HIV serologyif neededBefore starting an immunosuppressive treatment [2].
- IgA anti-tissue transglutaminase antibodies (tTG-IgA) (once, in the baseline tests of confirmed Sjögren disease)if needednon-French guidelineThe BSR (2024) recommends, at baseline in confirmed Sjögren's disease and then according to the clinical picture, looking for associated conditions, including coeliac disease by anti-transglutaminase antibodies: a Hungarian series found 4.5% in Sjögren's, versus 4.5 to 5.5 per 1 000 in the European population [4]. The initial work-up of the PNDS does not include this test [2]. The NABM lists this test as first-line in adults suspected of coeliac disease [6].
- 25-hydroxyvitamin D (once, in the baseline tests of confirmed Sjögren disease)if needednon-French guidelineThe BSR (2024) recommends a vitamin D level at baseline (strong recommendation, level of evidence B): deficiency is common beyond 40° of latitude and, in Sjögren's, associated with peripheral neuropathy and lymphoma [4]. The PNDS does not provide for it [2] and the NABM does not cover this test for this indication [6].
Do not order
- Follow-up anti-SSA/SSB antibodies: useless, their titre does not vary with activity [2].
Sources (6)
- Centre de référence des maladies auto-immunes et systémiques rares (CeRAINO, Île-de-France), filière FAI²R. Maladie (ou syndrome) de Sjögren : synthèse à destination du médecin traitant, extraite du PNDS. Haute Autorité de santé, mars 2022.
- Centre de référence des maladies auto-immunes et systémiques rares (CeRAINO, Île-de-France), filière FAI²R. Protocole national de diagnostic et de soins (PNDS) : maladie (ou syndrome) de Sjögren. Haute Autorité de santé, mars 2022.
- Assurance maladie. Nomenclature des actes de biologie médicale (NABM), version 105 : sous-chapitre 7-03 auto-immunité, actes 1456-1457 (anti-antigènes nucléaires solubles) et conditions de recherche des anti-SSA en l'absence d'antinucléaires.
- Price EJ, Benjamin S, Bombardieri M, et al. British Society for Rheumatology guideline on management of adult and juvenile onset Sjögren disease. Rheumatology (Oxford). 2025;64(2):409-439.
- Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren's Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts. Arthritis Rheumatol. 2017;69(1):35-45.
- Assurance maladie. Nomenclature des actes de biologie médicale (NABM), version 105 : actes 1139 (25-OH-vitamine D) et 1828 (anticorps antitransglutaminase IgA), conditions de prise en charge.