If the diagnosis is secondary Sjögren’s syndrome (sSS), the focus is on treating the underlying disease.
Therapy Objective
- Relief of symptoms
Therapy recommendations
Note: According to the recommendations of the European League Against Rheumatism (EULAR), patients with Sjögren’s syndrome should be treated in or or in close collaboration with specialized centers and multidisciplinary.
- Topical therapies (application of medical agents where they are intended to have a therapeutic effect) for symptomatic treatment:
- Eye dryness: tear substitutes: eye drops (artificial tears) and eye gels/eye ointments.
- Refractory/severe eye dryness: to promote tear production: ciclosporin (cyclosporin A) (topical application) and serum eye drops
- Dry mouth: nonpharmacologic stimulation for mild salivary gland dysfunction; pharmacologic stimulation for moderate dysfunction; salivary substitution for severe dysfunction [EULAR recommendation].
- Saliva substitutes (Artificial saliva) – They are intended to provide long-lasting moistening of the dental hard tissues as well as the oral mucosa.
- To increase the secretion of saliva and tears: bromhexine, pilocarpine (via an excitation of the parasympathetic nervous system occurs increased saliva production).
- Eye dryness: tear substitutes: eye drops (artificial tears) and eye gels/eye ointments.
- Systemic therapies for treatment in active systemic diseases:
- Musculoskeletal pain: analgesics (pain relievers) or other pain-modifying agents should be considered [EULAR recommendation].
- In patients with arthralgias (joint pain): hydroxychloroquine (leads to a reduction in hypergammaglobulinemia).
- Therapy with systemic immunosuppressants such as glucocorticoids is required only if internal organs are affected or vasculitis (inflammation of blood vessels) is present (see at the respective clinical picture)Note: Glucocorticoids should be used at the minimum dose and duration required to control active systemic disease [EULAR recommendation].
- Severe refractory systemic disease: B-cell-targeted therapies may be considered [EULAR recommendation]. These should be individualized according to the specific histologic subtype and disease stage.
- Sequential (or combined) therapy with glucocorticoids, immunosuppressants, and biologics is followed by the systemic organ-specific therapy approach [EULAR recommendation].
- In very severe courses or non-Hodgkin’s lymphoma: rituximab (see under “Non-Hodgkin’s lymphoma“).
Further notes
- The efficacy of conventional disease-modifying antirheumatic drugs (DMARDs) or biologics in Sjögren’s syndrome has not been established by randomized controlled trials.
