Sjögren syndrome is a systemic autoimmune disease that primarily affects the lacrimal and salivary glands, causing persistent ocular and oral dryness, fatigue, and inflammatory joint pain.

Although historically considered rare, it is now recognised as the most common systemic autoimmune disease, with a prevalence of 2%-4%.

Because early symptoms are nonspecific and presentations vary widely, timely recognition is essential to reduce the risk for systemic complications, including lymphoma.

Sjögren syndrome may be primary or occur alongside autoimmune conditions such as rheumatoid arthritis, systemic lupus erythematosus, or systemic sclerosis.

Lymphocytic infiltration of the exocrine glands results in xerophthalmia, xerostomia, and dryness of the skin, airways, and vagina.

The classic triad includes persistent dryness, chronic fatigue, and inflammatory joint pain. Xerophthalmia may impair visual acuity and increase the risk for corneal ulceration, whereas xerostomia predisposes patients to dental caries and oral candidiasis.

Clinical Features

Systemic involvement occurs in 30%-40% of individuals. Manifestations include parotitis, lymphadenopathy, splenomegaly, interstitial lung disease, distal renal tubular acidosis, peripheral neuropathy, and cutaneous features such as purpura and Raynaud phenomenon. The overall risk for lymphoma is approximately 5% and is higher in individuals with active systemic disease or immunologically active profiles.

The condition disproportionately affects women, with a female-to-male ratio of approximately 9:1, and typically presents at approximately 50 years of age.

Diagnostic Criteria

Diagnosis integrates clinical features with laboratory testing, functional gland assessment, and histology. The French National Authority for Health recommends:

Laboratory testing includes:

• Presence of antinuclear antibodies, particularly anti-SSA/Ro60 and anti-SSB
• Rheumatoid factor, present in about half of cases
• Polyclonal hypergammaglobulinaemia
• Complement evaluation, typically C4 or C3 and C4
• Cryoglobulinaemia when systemic involvement is suspected

Functional testing includes:

• Schirmer test: Measurement of tear production using blotting paper placed in the conjunctival fornix, with a result ≤ 5 mL in 5 minutes
• Ocular staining score using vital dyes such as fluorescein, with a score ≥ 5
• Unstimulated salivary flow measured over 15 minutes, with a flow rate of ≤ 0.1 mL/min

Histology includes:

• Minor salivary gland biopsy showing a focus score ≥ 1, defined as an infiltrate of at least 50 lymphocytes per 4 mm²

Activity scoring includes:

• The European League Against Rheumatism (EULAR) Sjögren Syndrome Patient Reported Index used to assess individually reported dryness, fatigue, and pain
• The EULAR Sjögren Syndrome Disease Activity Index used to evaluate systemic disease activity across organ domains

Individuals with an active immunological profile, notably those who are anti-SSA/Ro60- and anti-SSB-positive, are at a greater risk for systemic complications.

Finally, the classification is based on the American College of Rheumatology and EULAR 2016 criteria, which requires a score ≥ 4. A weighted score of 3 points is assigned for an objective immunological abnormality, defined as anti-SSA/Ro60 positivity or a minor salivary gland biopsy with a focus score ≥ 1.

Each positive functional test, including an ocular staining score ≥ 5, a Schirmer test ≤ 5 mm in 5 minutes, or an unstimulated salivary flow ≤ 0.1 mL/min, contributes to 1 point.

The exclusion criteria apply and include a history of cervical irradiation, active hepatitis C, HIV-associated disease, sarcoidosis, amyloidosis, graft-vs-host disease, or immunoglobulin G4-related disease.

Despite clear criteria, diagnosis remains challenging because the clinical presentations are heterogeneous, often lack early dryness, and commonly overlap with symptoms frequently seen in the general population.

Management and Monitoring

Management focuses on symptomatic relief and prevention of systemic complications.

Treatment depends on the clinical manifestations and includes:

• Dry eyes: Moisturising drops and artificial tears
• Dry mouth: Regular hydration, moisturising gels, saliva substitutes, bicarbonate mouth rinses, or pilocarpine as a compounded preparation to stimulate salivation
• Systemic involvement: Low-dose corticosteroids, typically 5-10 mg/d, hydroxychloroquine, or immunosuppressive therapies such as methotrexate or rituximab, depending on organ involvement

Sjögren syndrome requires regular oral and ophthalmological reviews supported by clinical and laboratory surveillance to assess disease activity, detect systemic involvement, and evaluate the risk for lymphoma.

The 2022 National Diagnostic and Treatment Protocol from the French National Authority for Health provides structured guidance for long-term follow-up care.

Treatment is multidisciplinary and coordinated according to the disease severity and progression. Care may be provided by clinicians in primary care with specialist input or by hospital specialists in more severe cases. Referral to expert centres is recommended for refractory, severe, or multisystem forms.

The FAI²R health network, dedicated to rare autoimmune and autoinflammatory diseases, brings together reference centres and competence centres to support the coordinated management of Sjögren syndrome across regions.

Patient education programs are an essential part of long-term care, helping individuals manage their conditions more effectively on a day-to-day basis.

Support from organisations such as the French Association for Sjögren’s Syndrome and Dry Syndromes further improves access to information and community resources.

This story was translated from Univadis France.