Treatment of Sjögren's Syndrome and Associated Small Fiber Neuropathy

General Management Principles

The European League Against Rheumatism (EULAR) recommends a multidisciplinary approach for the treatment of Sjögren's syndrome with small fiber neuropathy, focusing on symptomatic relief of sicca symptoms and targeted therapy for neuropathic manifestations, with systemic therapies reserved for active systemic disease 1, 2
Patients with Sjögren's syndrome should be managed at, or in close collaboration with, centers of expertise following a multidisciplinary approach involving rheumatologists, neurologists, ophthalmologists, and oral health specialists 2
The first therapeutic approach for dryness symptoms should be symptomatic relief using topical therapies 2
Systemic therapies should be considered for the treatment of active systemic disease, including neuropathic manifestations 2

Baseline evaluation of salivary gland function is recommended before starting treatment 2
Treatment should be tailored according to salivary gland function, with non-pharmacological stimulation for mild dysfunction, pharmacological stimulation with muscarinic agonists for moderate dysfunction 3, and saliva substitution for severe dysfunction 2
First-line therapy for ocular dryness includes artificial tears and ocular gels/ointments 2
For refractory/severe ocular dryness, consider topical immunosuppressive-containing drops and autologous serum eye drops 2

Second-line agents for symptomatic treatment of neuropathic pain include analgesics according to pain severity, following the WHO pain ladder 4
Rituximab may be considered in patients with severe, refractory systemic disease including neuropathic manifestations 2

Treatment of systemic manifestations should be tailored to organ-specific severity using the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) definitions 2, 5
Glucocorticoids should be used at the minimum dose and length of time necessary to control active systemic disease 2
Immunosuppressive agents should be mainly used as glucocorticoid-sparing agents 2
B-cell targeted therapies, particularly rituximab, may be considered in patients with severe, refractory systemic disease 2

Regular assessment of disease activity using validated tools, such as the ESSDAI for systemic activity, is recommended 2
Monitoring of treatment response and side effects at regular intervals is necessary 2
Evaluation for development of lymphoma, which occurs in 2-5% of Sjögren's patients, is important 3