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autoimmune neuropathy
ICD-10 Codes
Description
Autoimmune neuropathies are a group of conditions in which the body's immune system mistakenly attacks and damages its own peripheral nerves, leading to various symptoms and complications.
Causes and Risk Factors
- Autoimmune diseases such as Sjogren’s syndrome, lupus, rheumatoid arthritis, and celiac disease can increase the risk of developing autoimmune neuropathy [1][2].
- In some cases, the immune system may attack the autonomic nervous system, leading to conditions like autoimmunue autonomic ganglionopathy (AAG) [3].
Symptoms
- Symptoms of autoimmune neuropathy can vary depending on the specific condition and affected nerves.
- Common symptoms include:
- Numbness or tingling sensations in hands and feet
- Muscle weakness or paralysis
- Abnormal sensitivity to touch or temperature
- Autonomic dysfunction, such as changes in heart rate, blood pressure, or digestion [4][5].
Types of Autoimmune Neuropathy
- There are several types of autoimmune neuropathies, including:
- Guillain-Barré syndrome: a rare and rapidly progressing condition that can affect autonomic nerves
- Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): a condition characterized by inflammation and damage to nerve roots [6].
Complications
- Untreated or poorly managed autoimmune neuropathy can lead to significant morbidity and mortality, including:
- Muscle atrophy and weakness
- Autonomic dysfunction and related complications
- Increased risk of falls and injuries due to muscle weakness and balance problems [7][8].
It's essential to consult a healthcare professional for an accurate diagnosis and treatment plan if you suspect you may have autoimmune neuropathy.
Additional Characteristics
- Muscle weakness or paralysis
- Numbness or tingling sensations in hands and feet
- Abnormal sensitivity to touch or temperature
- Autonomic dysfunction, such as changes in heart rate, blood pressure, or digestion
- Muscle atrophy and weakness
- Autonomic dysfunction and related complications
- Increased risk of falls and injuries due to muscle weakness and balance problems
Signs and Symptoms
Autoimmune neuropathies are a group of conditions where the body's immune system mistakenly attacks its own nerves, leading to various symptoms. Here are some common signs and symptoms:
- Numbness or tingling: Many people experience numbness or tingling sensations in their feet or hands, which can be a gradual onset or sudden (1, 3).
- Pain: Sharp, jabbing, throbbing, or burning pain can occur in the affected areas, making it difficult to perform daily activities (2, 8).
- Muscle weakness: Muscle weakness or paralysis can also occur, affecting various parts of the body (3, 9).
- Digestive problems: Some individuals may experience urinary problems, sexual difficulties, and difficulty digesting food due to nerve damage (1).
- Autonomic changes: Autonomic neuropathy can cause skin color changes, swelling, changes in pupil size, blurry vision, and abnormal heart rate or rhythm (5, 6, 7).
It's essential to note that the symptoms of autoimmune neuropathies can vary widely from person to person and may progress gradually over time. If you're experiencing any of these symptoms, it's crucial to consult a healthcare professional for proper diagnosis and treatment.
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Additional Symptoms
- Digestive problems
- Numbness or tingling
- Autonomic changes
- muscle weakness
- pain
Diagnostic Tests
Autoimmune neuropathies are a group of conditions in which the immune system attacks the nerves, leading to various symptoms such as numbness, weakness, and pain. Diagnosing autoimmune neuropathy can be challenging, but several diagnostic tests can help confirm the condition.
Diagnostic Tests for Autoimmune Neuropathy
- Electrophysiology tests: These tests measure the electrical activity of the nerves and can help identify abnormalities in nerve function.
- Blood tests: Blood tests can detect specific antibodies associated with autoimmune neuropathies, such as Guillain-Barré syndrome (GBS) or chronic inflammatory demyelinating polyneuropathy (CIDP).
- Imaging studies: Imaging studies like MRI scans and CT scans can help rule out other conditions that may be causing the symptoms.
- Nerve conduction study: This test measures the speed and strength of electrical signals in the nerves.
- Electromyogram: This test records the electrical activity of muscles to determine if there is any nerve damage.
Other Diagnostic Tests
- Urinalysis and bladder function tests: These tests can help evaluate bladder function and rule out other conditions that may be causing symptoms.
- 24-hour urine heavy metal test: This test can help diagnose heavy metal toxicity, which can cause neuropathy.
- Skin biopsy: In some cases, a skin biopsy may be performed to rule out other conditions that may be causing symptoms.
Diagnostic Algorithms
Evidence-based guidelines and diagnostic algorithms have been published for specific neuropathic phenotypes such as distal symmetric peripheral neuropathy, small fiber neuropathy, or inflammatory neuropathies. However, these guidelines are only applicable for specific neuropathic conditions or when a specific diagnosis is already suspected on clinical grounds.
References
- [1] Antibody testing is not part of accepted diagnostic criteria or management strategies for autoimmune neuropathies.
- [3] Diagnosis requires a comprehensive history, physical examination, and judicious laboratory testing.
- [5] The presence of an autoantibody in a patient does not assure a diagnosis of an autoimmune disease.
- [11] Serum autoantibodies are markers for a variety of immune-mediated disorders, which can be valuable in aiding diagnosis and helping physicians treat disorders with optimal, appropriate therapies.
- [12] Evidence-based guidelines and diagnostic algorithms have been published for specific neuropathic phenotypes.
Additional Diagnostic Tests
- Blood tests
- Skin biopsy
- Imaging studies
- Electrophysiology tests
- Nerve conduction study
- Electromyogram
- Urinalysis and bladder function tests
- 24-hour urine heavy metal test
Treatment
Autoimmune neuropathies are a group of conditions where the immune system attacks the nerves, leading to various symptoms such as pain, numbness, and weakness. While there is no cure for these conditions, several drug treatments can help manage the symptoms and slow down disease progression.
First-line immunotherapies
- Intravenous immunoglobulin (IVIG) [5]
- IVIG involves infusing antibodies into the bloodstream to suppress the immune system's attack on the nerves.
- It has been shown to be effective in treating autoimmune neuropathies, particularly those caused by anti-MAG antibodies [2].
- Plasma exchange
- This treatment involves removing plasma from the blood and replacing it with a solution that contains antibodies against the immune system's attack on the nerves.
- Plasma exchange can help reduce inflammation and slow down disease progression [6].
- Corticosteroids
- These medications, such as prednisone, can help suppress the immune system's attack on the nerves.
- However, long-term use of corticosteroids can have significant side effects, so they are typically used in combination with other treatments [5].
Other treatment options
- Rituximab
- This medication targets B lymphocytes, which play a key role in autoimmune neuropathies [2].
- Rituximab has been shown to be effective in treating autoimmune neuropathies, particularly those caused by anti-MAG antibodies.
- High-dose steroids or chronic steroid therapy
- These treatments can help reduce inflammation and slow down disease progression [3].
Pain management
- Topical patches containing lidocaine or an anti-epileptic or antidepressant medication may be prescribed to manage chronic nerve-related pain [4].
It's essential to note that each individual's response to treatment may vary, and the most effective treatment plan will depend on the specific type of autoimmune neuropathy being treated. Consultation with a healthcare professional is necessary to determine the best course of treatment.
References: [1] Not applicable [2] C Briani (2022) - Rituximab in the treatment of anti-MAG antibody neuropathy and autoimmune autonomic neuropathy. [3] Mar 19, 2019 - Alternative therapies for patients with autoimmune autonomic neuropathy. [4] For people with chronic nerve-related pain, a doctor may prescribe topical patches containing lidocaine or an anti-epileptic or antidepressant medication. [5] A Sivadasan (2022) - Autoimmune neuropathies: Treatment options and outcomes. [6] JR Schofield (2018) - Immunoglobulin therapy in the treatment of autoimmune forms of dysautonomia.
Recommended Medications
- Corticosteroids
- Plasma exchange
- Intravenous immunoglobulin (IVIG)
- Rituximab
- High-dose steroids or chronic steroid therapy
- Topical patches containing lidocaine or an anti-epileptic or antidepressant medication
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Autoimmune neuropathies are a group of conditions where the immune system mistakenly attacks and damages the nerves, leading to various symptoms. The differential diagnosis of autoimmune neuropathy involves identifying the underlying cause of the condition.
Possible Causes:
- Sjogren's syndrome: This is an autoimmune disease that can affect the nerves, causing peripheral neuropathy [6].
- Lupus: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can cause peripheral neuropathy [6].
- Rheumatoid arthritis: This condition can also be associated with peripheral neuropathy, although it is less common [6].
Other Conditions to Consider:
- Guillain-Barré syndrome: This is an autoimmune disorder that can cause rapid-onset muscle weakness and paralysis [5].
- Chronic inflammatory demyelinating polyneuropathy (CIDP): This condition is characterized by progressive muscle weakness, numbness, and tingling in the hands and feet [5].
Key to Differential Diagnosis:
The key to differential diagnosis of peripheral neuropathies presenting with autonomic dysfunction lies in a combination of detailed history taking, physical examination, and laboratory tests [8]. A thorough medical history, including symptoms, duration, and progression of the condition, is essential for making an accurate diagnosis.
References:
- [6] Sjogren's syndrome, lupus, and rheumatoid arthritis are among the autoimmune diseases that can be associated with peripheral neuropathy.
- [8] The key to the differential diagnosis of peripheral neuropathies presenting with autonomic dysfunction (Table 1) is the combination of detailed history taking, physical examination, and laboratory tests.
Additional Differential Diagnoses
- Lupus
- Chronic inflammatory demyelinating polyneuropathy (CIDP)
- N syndrome
- Sjogren's syndrome
- rheumatoid arthritis
- acrofacial dysostosis, Patagonia type
Additional Information
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- An autoimmune disease of central nervous system caused by an autoimmune response.
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