Who treats neuropathy caused by Sjogren's syndrome, a rheumatologist or a neurologist?

Management of Neuropathy in Sjögren's Syndrome: A Multidisciplinary Approach

Both rheumatologists and neurologists should be involved in treating neuropathy caused by Sjögren's syndrome, with the rheumatologist managing the underlying autoimmune disease and the neurologist addressing the specific neuropathic manifestations. 1

Rationale for Multidisciplinary Management

• Sjögren's syndrome is a systemic autoimmune disease that primarily affects exocrine glands but can cause various "extraglandular" complications, with peripheral neuropathies being among the most common and severe systemic manifestations 2
• The EULAR recommendations explicitly state that "a multidisciplinary approach involving various health professionals is essential" for Sjögren's syndrome management, with specialists in autoimmune diseases acting as coordinators of the healthcare process 1
• The complexity of neurological manifestations in Sjögren's syndrome requires specialized neurological expertise for proper diagnosis and treatment 2, 3

Types of Neuropathies in Sjögren's Syndrome

• Sensory neuropathies are the most frequent peripheral nervous system complication in Sjögren's syndrome 2, 3
• Small-fiber neuropathy causing lancinating or burning pain, often in a "non-length-dependent distribution" 2
• Sensory ataxic neuropathy (ganglionopathy) affecting proprioceptive large fibers 3
• Sensorimotor polyneuropathy affecting both sensory and motor axons 3
• Rare types including autoimmune demyelinating neuropathy, mononeuropathy, mononeuropathy multiplex, and autonomic neuropathy 3
• Carpal tunnel syndrome is also common, affecting up to 54% of Sjögren's patients with neuropathy 4

Diagnostic Approach

• Skin biopsy assessing intraepidermal nerve fiber density is useful for diagnosing small-fiber neuropathies 2
• Electrodiagnostic studies (nerve conduction studies and electromyography) are essential for evaluating polyneuropathy 1, 3
• Additional testing may include MRI of the spine/brain, lumbar puncture, and autoimmune panels 1
• Neurological symptoms may precede the diagnosis of Sjögren's syndrome in some patients, highlighting the importance of considering autoimmune causes in unexplained neuropathies 4

Treatment Approach

Systemic Management (Rheumatologist-led)

• Treatment of systemic disease should be tailored to organ-specific severity using the ESSDAI definitions 1
• For neuropathies associated with Sjögren's syndrome, treatment follows a sequential approach:
  • Glucocorticoids at the minimum dose and duration necessary to control active systemic disease 1
  • Immunosuppressive agents (cyclophosphamide, azathioprine, methotrexate, leflunomide, mycophenolate) as glucocorticoid-sparing agents 1
  • B-cell targeted therapies (rituximab) may be considered in patients with severe, refractory systemic disease 1

Neuropathy-Specific Management (Neurologist-led)

• Symptomatic treatment of neuropathic pain with agents such as pregabalin, gabapentin, or duloxetine 1
• Intravenous immunoglobulins (IVIG) have shown benefit in 82% of treated patients with peripheral neuropathies in Sjögren's syndrome 5
• Cyclophosphamide has shown good results specifically for mononeuritis multiplex 5
• Autonomic neuropathies have responded well to glucocorticoids or rituximab 5
• For sensory neuronopathy, corticosteroids in association with immunosuppressive drugs (particularly mycophenolate mofetil) have shown positive results 6

Treatment Considerations Based on Neuropathy Type

• Small-fiber neuropathy: Symptomatic treatment with neuropathic pain medications; consider IVIG or immunosuppressants if severe 2, 5
• Sensory ataxic neuropathy: More aggressive immunosuppression often required; consider mycophenolate mofetil 6
• Sensorimotor polyneuropathy: Often associated with systemic manifestations; may require combination therapy with glucocorticoids and immunosuppressants 3
• Mononeuritis multiplex: Cyclophosphamide has shown good results 5

Practical Considerations

• The progression of Sjögren's-related sensory neuronopathy tends to be chronic, insidious, and potentially debilitating despite treatment 6
• Neurological manifestations may be the presenting feature of Sjögren's syndrome in some patients, emphasizing the importance of autoimmune screening in patients with unexplained neuropathy 4
• Patients with Sjögren's syndrome often suffer from fatigue, sicca symptoms, and autonomic findings, which can be worsened by certain neuropathic medications 2
• Treatment decisions should be made in a multidisciplinary setting with input from both rheumatology and neurology specialists 1

Pitfalls and Caveats

• Neurological symptoms may precede other manifestations of Sjögren's syndrome, potentially leading to delayed diagnosis if autoimmune causes are not considered 4
• The evidence base for treatment of Sjögren's-related neuropathies consists primarily of case reports and small case series, with no large randomized controlled trials 5
• Intravenous immunoglobulins, while effective in many cases, had disappointing results specifically for sensory neuronopathy 6
• Patients may require long-term follow-up as the neuropathy can progress despite treatment 6