From the Guidelines
Systemic sclerosis patients with neurologic symptoms should be managed with immunosuppressants and symptom-specific treatments to improve morbidity, mortality, and quality of life. The management of systemic sclerosis (SSc) involves non-pharmacological and pharmacological interventions, with the European Alliance of Associations for Rheumatology (EULAR) providing recommendations for the pharmacological management of SSc 1. Patients with SSc may experience various neurological symptoms, including peripheral neuropathy, trigeminal neuralgia, headaches, cognitive impairment, and autonomic dysfunction, which occur due to vascular damage, immune-mediated inflammation, and fibrosis affecting neural tissues.
Neurological Complications
The neurological complications of SSc can be managed with symptom-specific treatments, such as:
- Gabapentin (300-1200mg three times daily) or pregabalin (75-300mg twice daily) for neuropathic pain
- Carbamazepine (200-400mg twice daily) for trigeminal neuralgia
- Midodrine (2.5-10mg three times daily) for orthostatic hypotension Regular neurological assessments are essential for early detection and management of these complications, as they can indicate disease progression or medication side effects 1.
Treatment Recommendations
The EULAR recommendations for the treatment of SSc have been updated in 2023, providing 22 recommendations for the management of SSc symptoms and organ involvement, including Raynaud’s phenomenon, digital ulcers, pulmonary arterial hypertension, scleroderma renal crisis, skin fibrosis, interstitial lung disease, gastrointestinal, and musculoskeletal manifestations 1. The use of immunosuppressants, such as mycophenolate mofetil (1-2g daily), cyclophosphamide (1-2mg/kg/day), or rituximab (two 1g infusions two weeks apart), is recommended for managing the underlying systemic sclerosis.
Quality of Life
Physical therapy and occupational therapy can help maintain function and independence in patients with SSc, improving their quality of life. Patients should report new or worsening neurological symptoms promptly, as they may indicate disease progression or medication side effects. The management of SSc requires a multidisciplinary approach, with regular assessments and adjustments to treatment as needed to improve morbidity, mortality, and quality of life 1.
From the Research
Systemic Sclerosis and Neurologic Symptoms
- Systemic sclerosis (SSc) is a complex autoimmune disease that can affect various parts of the body, including the nervous system 2.
- Neurologic involvement in SSc can manifest in different ways, including central nervous system (CNS) involvement, peripheral nervous system (PNS) involvement, and autonomic nervous system (ANS) involvement 3.
- CNS involvement in SSc can include symptoms such as headache, seizures, and cognitive impairment, while PNS involvement can include symptoms such as myopathy, trigeminal neuropathy, and peripheral sensorimotor polyneuropathy 3.
- ANS involvement in SSc can include symptoms such as autonomic neuropathy, which can affect various systems, including the cardiovascular and gastrointestinal systems 3.
- Treatment of neurologic symptoms in SSc can vary depending on the specific symptoms and severity of the disease, but may include medications such as corticosteroids and cyclophosphamide 3, 4.
- Immunotherapy, including traditional immunosuppressants and biological agents, may also be effective in treating SSc, including its neurologic symptoms 5.
Neurologic Involvement in SSc
- A systematic review of neurologic involvement in SSc found that CNS involvement was characterized by headache, seizures, and cognitive impairment, while PNS involvement was characterized by myopathy, trigeminal neuropathy, and peripheral sensorimotor polyneuropathy 3.
- The review also found that ANS involvement was common in SSc, and could affect various systems, including the cardiovascular and gastrointestinal systems 3.
- Another study found that treatment of neurologic symptoms in SSc can be challenging, and may require a multidisciplinary approach 4.
Treatment of Neurologic Symptoms in SSc
- Corticosteroids and cyclophosphamide are commonly used to treat neurologic symptoms in SSc, particularly in severe cases 3, 4.
- Immunotherapy, including traditional immunosuppressants and biological agents, may also be effective in treating SSc, including its neurologic symptoms 5.
- Autologous stem cell transplantation has shown promise in treating SSc, including its neurologic symptoms, although it is a high-risk procedure 5.