Sjögren Syndrome
Sjögren syndrome is a chronic, slowly progressive systemic autoimmune disease in which lymphocytes (mainly CD4+ T cells and B cells) infiltrate the body's exocrine glands. This long-standing infiltration and inflammation destroys the functional structure of the glands and sharply reduces their secretions. The lacrimal and salivary glands are hit hardest, producing the classic sicca symptoms: dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia).
In clinical practice at Haute-Derma, we classify the disease into two forms:
- Primary Sjögren's: Occurs on its own, without any other systemic autoimmune disease.
- Secondary Sjögren's: Develops alongside an existing autoimmune connective tissue disease, most often rheumatoid arthritis, systemic lupus erythematosus (SLE) or scleroderma.
Although its most obvious effects are on the mucous membranes, Sjögren's is fundamentally a systemic disease. Circulating autoantibodies such as anti-SSA (Ro) and anti-SSB (La) are linked to extraglandular manifestations such as vasculitis, interstitial lung fibrosis, interstitial nephritis and peripheral neuropathy. Most importantly, the disease raises the risk of a lymphoid malignancy (B-cell lymphoma) 15 to 40 times above that of the general population.
Our Approach to Treating Sjögren Syndrome
At Haute-Derma, we manage Sjögren syndrome with a whole-person approach that brings together dermatology, ophthalmology and rheumatology:
- Comprehensive protection of the mucous membranes: Going beyond surface treatments, we focus on restoring the natural moisture barrier and lipid layer of the skin, eyes and mouth to prevent infection and dryness-related erosions and ulcers.
- Screening for systemic involvement and malignancy: We set up regular follow-up to detect interstitial lung disease, cutaneous vasculitis and, above all, early warning signs of lymphoma (such as swollen neck nodes or persistent parotid enlargement).
- Personalized immunomodulatory therapy: We work with rheumatologists to design targeted treatment that keeps autoantibody levels in check while preserving as much of your natural immune function as possible.
How We Diagnose Sjögren Syndrome at Haute-Derma
To diagnose Sjögren syndrome accurately according to the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) consensus criteria, Haute-Derma uses a multi-step workup:
Symptom Review and Clinical Examination
- The doctor asks about dry eyes and dry mouth lasting more than 3 months (a gritty, sandy feeling in the eyes; needing to sip water to swallow dry food).
- Examination of the face and jaw: We check for enlargement of the parotid or submandibular glands (on both sides or one), rapidly progressing decay at the gum line, and secondary oral candidiasis.
- Skin assessment: We look for palpable purpura from vasculitis and for rough, generalized dry skin (xerosis) caused by reduced sweat gland output.
Tests of Exocrine Gland Function
- Schirmer's test: A strip of special filter paper is placed at the lower eyelid to measure tear production over 5 minutes. The result is positive (reduced tears) when 5 mm or less of the strip is wetted.
- Ocular surface staining: Lissamine green or fluorescein dye reveals areas of the corneal and conjunctival surface that are damaged or shedding because the protective tear film is missing.
- Unstimulated whole salivary flow: The patient collects and spits saliva into a tube for 15 minutes. A flow rate of 0.1 mL/min or less indicates reduced salivary gland function.
Immunology Blood Tests (Serology)
Specific autoantibodies are measured in the blood by ELISA:
- Anti-SSA (Ro): The most valuable diagnostic marker, found in about 60–70% of patients with primary disease.
- Anti-SSB (La): Usually found together with anti-SSA.
- Rheumatoid factor (RF) and antinuclear antibodies (ANA): Often positive at high titers.
Minor Salivary Gland Biopsy
This is the most objective and most specific diagnostic test:
- In a minor procedure, the doctor removes 4–5 minor salivary gland lobules from the inner surface of the lower lip.
- Histological assessment (focus score): The sample is stained and examined under the microscope. The diagnosis is confirmed by finding foci of inflammatory cells (each with 50 or more lymphocytes) around the gland ducts, with a focus score of ≥ 1 per 4 mm² of glandular tissue.
How Sjögren Syndrome Is Treated at Haute-Derma
Treatment of Sjögren syndrome at Haute-Derma is an ongoing process that combines local symptom replacement with systemic immunomodulation to prevent organ damage:
Local Control (Replacing and Stimulating Secretions)
- Eye care and protection: Preservative-free artificial tears (preferably high-viscosity gels, or an ointment at night) rebuild the tear film. In severe cases, we work with an ophthalmologist to place punctal plugs that keep the limited natural tears on the eye surface.
- Oral care: Saliva substitutes and dedicated alcohol-free moisturizing mouthwashes. Oral secretagogues (such as pilocarpine or cevimeline) are used if the glands still have some function left.
- Whole-body skin care: Moisturizers rich in ceramides and hyaluronic acid, plus lipid-restoring care right after bathing, help make up for reduced sweat gland function.
Systemic Treatment and Immunomodulation
When extraglandular features appear (arthritis, vasculitis, lung or nerve involvement), systemic treatment is required:
- Antimalarials (hydroxychloroquine): The cornerstone of treatment for systemic Sjögren's. It modulates the immune response and lowers autoantibody levels, easing joint aches, persistent fatigue and skin involvement.
- Corticosteroids and immunosuppressants: High-dose oral or intravenous corticosteroids, combined with immunosuppressants (such as azathioprine, methotrexate or mycophenolate mofetil), are used for acute flares with interstitial lung disease, nephritis or systemic vasculitis.
- Targeted biologic therapy: In treatment-resistant cases or where there is a risk of lymphoma, anti-CD20 monoclonal antibodies (such as rituximab) may be considered to eliminate overactive B cells and interrupt the systemic autoimmune cascade.
