From the Guidelines
Intravenous immunoglobulin (IVIG) is a viable treatment option for neuropathies associated with Sjögren's syndrome, particularly for sensory ataxic neuropathy, small fiber neuropathy, and painful sensory neuropathy, as evidenced by the EULAR recommendations for the management of Sjögren's syndrome 1.
Key Considerations
- IVIG is typically administered at doses of 0.4–2 g/kg over 5 days, as outlined in the EULAR guidelines 1.
- The treatment response should be monitored regularly, with continuation based on documented clinical improvement.
- Before starting IVIG, patients should undergo baseline renal function testing, as the treatment carries risks including thromboembolic events, aseptic meningitis, and renal dysfunction.
Mechanism of Action
- IVIG provides anti-idiotypic antibodies, blocks Fc receptors on macrophages, inhibits complement-mediated damage, and modulates cytokine production.
Comparison with Other Treatments
- IVIG and plasma exchange are equally effective treatments for Guillain-Barré syndrome, with IVIG being generally easier to administer and more widely available 1.
- Corticosteroids have not been shown to be effective in the treatment of Guillain-Barré syndrome and may even have a negative effect on outcome 1.
Clinical Decision-Making
- The decision to use IVIG for neuropathies associated with Sjögren's syndrome should be made on a case-by-case basis, taking into account the individual patient's response to conventional treatments and the potential risks and benefits of IVIG therapy.
- The EULAR guidelines provide a framework for the therapeutic approach to patients with primary Sjögren's syndrome presenting with organ-specific systemic involvements, including the use of IVIG for neuropathies 1.
From the Research
Efficacy of IVIG in Sjögren's Syndrome and Neuropathies
- IVIG has been shown to be effective in treating painful sensory neuropathy associated with Sjögren's syndrome, with a significant reduction in neuropathic pain observed in patients treated with IVIG 2.
- A systematic review of immunosuppressive treatment for peripheral neuropathies in Sjögren's syndrome found that IVIG was useful in sensory, motor, and sensorimotor neuropathies, with 82% of patients treated with IVIG showing improvement 3.
- A national multicentric retrospective study found that IVIG therapy was effective in improving or stabilizing symptoms in patients with Sjögren's syndrome-related peripheral neuropathy, particularly those with sensorimotor or nonataxic sensory neuropathy 4.
- Case reports have also demonstrated the efficacy of IVIG in treating small fiber neuropathy associated with primary Sjögren's syndrome, with significant improvement in symptoms observed after treatment 5.
- Additionally, IVIG has been shown to be effective in treating ataxic sensory neuronopathy with Sjögren's syndrome, with remarkable improvement observed in four out of five patients treated 6.
Types of Neuropathies Responding to IVIG
- Sensorimotor neuropathy: IVIG has been shown to be effective in improving or stabilizing symptoms in patients with sensorimotor neuropathy associated with Sjögren's syndrome 4.
- Nonataxic sensory neuropathy: IVIG has been shown to be effective in improving or stabilizing symptoms in patients with nonataxic sensory neuropathy associated with Sjögren's syndrome 4.
- Small fiber neuropathy: IVIG has been shown to be effective in treating small fiber neuropathy associated with primary Sjögren's syndrome, with significant improvement in symptoms observed after treatment 5.
- Ataxic sensory neuronopathy: IVIG has been shown to be effective in treating ataxic sensory neuronopathy with Sjögren's syndrome, with remarkable improvement observed in four out of five patients treated 6.