Overview
Primary Sjögren's syndrome (pSS) is a chronic autoimmune disorder characterized by lymphocytic infiltration of exocrine glands, leading to symptoms like dry eyes and mouth, and can involve neurological manifestations including peripheral neuropathy 1.Diagnosis
Key Diagnostic Criteria: Presence of sicca symptoms, positive anti-SSA/Ro or anti-SSB/La antibodies, and lymphocytic infiltration in minor salivary glands or other characteristic features 1.
Recommended Tests: Salivary flow tests, Schirmer's test, parotid gland ultrasonography, and serological markers (anti-SSA/Ro, anti-SSB/La antibodies) 1.
Neurological Evaluation: Nerve conduction studies and electromyography for peripheral neuropathy assessment 1.Management
First-Line Treatments:
- Immunosuppressive Agents: Cyclophosphamide, mycophenolate mofetil (MMF) 1.
- Corticosteroids: Prednisone for acute exacerbations 1.
Adjunctive Treatments:
- Symptomatic Relief: Pilocarpine for dry mouth, artificial tears for dry eyes 1.
- Neuropathy Management: Gabapentin or pregabalin for neuropathic pain 1.Special Populations
Elderly: Increased risk of peripheral neuropathy; careful monitoring and management of neuropathic symptoms 1.
Comorbidities: Presence of comorbidities like age and pre-existing neurological conditions may increase risk of neuropathy 1.Key Recommendations
Screen for Peripheral Neuropathy: Regular neurological evaluations, particularly nerve conduction studies, in pSS patients, especially those older than 59 years (Evidence: Moderate) 1.
Consider Early Immunosuppressive Therapy: Initiate immunosuppressive agents like MMF or cyclophosphamide in patients with significant neurological involvement (Evidence: Moderate) 1.
Manage Symptomatic Neuropathy: Use gabapentin or pregabalin for neuropathic pain associated with pSS-related peripheral neuropathy (Evidence: Moderate) 1.References
1 Liampas A, Parperis K, Erotocritou MF, Nteveros A, Papadopoulou M, Moschovos C et al.. Primary Sjögren syndrome-related peripheral neuropathy: A systematic review and meta-analysis. European journal of neurology 2023. link