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autoimmune disease of peripheral nervous system

ICD-10 Codes

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Description

Autoimmune diseases of the peripheral nervous system (PNS) are a group of disorders in which the body's immune system attacks and damages the nerves outside the brain and spinal cord. This can lead to a wide range of symptoms, including muscle weakness, numbness or tingling sensations, pain, and paralysis.

Some examples of autoimmune diseases that affect the PNS include:

  • Guillain-Barré syndrome: a rare but serious condition in which the immune system attacks the nerves, leading to muscle weakness and paralysis [2].
  • Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): a condition in which the immune system attacks the myelin sheaths around nerve cells, leading to muscle weakness and numbness or tingling sensations [7].
  • Myasthenia gravis: an autoimmune disease that affects the nerves that control muscles, leading to muscle weakness and fatigue [2].

These conditions can be caused by a variety of factors, including genetic predisposition, environmental triggers, and other underlying medical conditions. Treatment options for autoimmune diseases of the PNS typically involve medications such as corticosteroids and immunosuppressants to reduce inflammation and suppress the immune system's attack on the nerves.

It is worth noting that autoimmune diseases of the PNS can be associated with high clinical disability [4][8]. Early diagnosis and treatment are crucial in managing these conditions and preventing long-term damage to the nervous system.

Additional Characteristics

  • Myasthenia gravis
  • Guillain-Barré syndrome
  • Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)

Signs and Symptoms

Autoimmune diseases affecting the peripheral nervous system can manifest in various ways, depending on the specific condition. Here are some common signs and symptoms:

  • Weakness and tingling: Weakness or numbness in the arms and legs is a hallmark symptom of conditions like Guillain-Barré syndrome (GBS) [1]. This weakness can progress to involve other areas of the body.
  • Loss of tendon reflexes: In GBS, loss of tendon reflexes in the arms and legs is another characteristic feature [3].
  • Muscle tenderness or pain: Muscle cramps, tenderness, or pain may be experienced by individuals with autoimmune diseases affecting the peripheral nervous system [3].
  • Sensation changes: Tingling, numbness, or a mild loss of sensation can occur in various parts of the body, including the hands and feet [4], [5].
  • Fever, fatigue, headache, nausea, vomiting: These symptoms are often associated with autoimmune diseases like GBS [2] and may be accompanied by more severe complications such as seizure and coma.
  • Swallowing or breathing problems: In some cases, individuals may experience difficulties swallowing (dysphagia) or breathing (respiratory failure), which can be life-threatening [7].
  • Fainting or low blood pressure: Autoimmune diseases affecting the autonomic nervous system, such as Acute Autonomic Ganglionopathy (AAG), can cause fainting, low blood pressure upon standing, and gastrointestinal symptoms [9].

It's essential to note that these symptoms can vary in severity and may be similar to those experienced by individuals with other conditions. A proper diagnosis by a healthcare professional is necessary for an accurate assessment and effective treatment.

References: [1] Context result 1 [2] Context result 2 [3] Context result 3 [4] Context result 4 [5] Context result 5 [7] Context result 7 [9] Context result 9

Additional Symptoms

Diagnostic Tests

Autoimmune diseases can affect various parts of the body, including the peripheral nervous system (PNS). Diagnosing these conditions requires a combination of medical history, physical examination, and laboratory tests.

Blood Tests Blood tests are often used to detect low levels of vitamins, diabetes, signs of inflammation, or metabolic issues that can cause peripheral neuropathy [6]. Autoantibody testing can be considered in individuals presenting with severe or rapidly progressive muscle weakness and sensory symptoms and no known underlying cause [4].

Immunofluorescence Immunofluorescence is a useful initial screening test for autoimmune diseases, such as Systemic Lupus Erythematosus (SLE) and Sjögren's syndrome [1]. However, it may not be specific enough to diagnose peripheral neuropathy.

Nerve Conduction Studies and Electromyography In most situations, nerve conduction studies and electromyography are crucial in distinguishing between primary axonal, primary demyelinating, or mixed axonal and demyelinating neuropathies [8]. These tests can help identify the type of peripheral neuropathy and guide further management.

Targeted Phenotype-Specific Panels Targeted phenotype-specific panels are preferred for evaluating suspected autoimmune disease of the central and peripheral nervous system [3].

Autoantibody Testing Autoantibody testing, such as anti-myelin-associated glycoprotein (MAG) IgM and anti-disialosyl IgM antibodies, can be key in diagnosing specific types of neuropathy, like anti-MAG neuropathy [5].

Diagnostic Approach The diagnostic approach for autoimmune diseases affecting the PNS typically involves a combination of medical history, physical examination, blood tests, nerve conduction studies, electromyography, and targeted phenotype-specific panels. A thorough evaluation by a neurologist or other specialist is essential to determine the best course of action.

References: [1] C Castro (2010) - Immunofluorescence as an initial screening test for autoimmune diseases. [4] Anonymous (2024) - Autoantibody testing in individuals with severe muscle weakness and sensory symptoms. [5] E Pascual-Goñi (2024) - Anti-myelin-associated glycoprotein (MAG) IgM and anti-disialosyl IgM antibodies in neuropathy diagnosis. [6] Anonymous (2023) - Blood tests for peripheral neuropathy. [7] S Bhagavati (2021) - Autoimmune disorders of the nervous system. [8] Anonymous (2024) - Nerve conduction studies and electromyography in distinguishing between primary axonal, primary demyelinating, or mixed axonal and demyelinating neuropathies.

Additional Diagnostic Tests

  • Blood Tests
  • Autoantibody Testing
  • Immunofluorescence
  • Nerve Conduction Studies and Electromyography
  • Targeted Phenotype-Specific Panels

Treatment

Autoimmune diseases affecting the peripheral nervous system (PNS) can be challenging to treat, but various medications have shown promise in managing these conditions.

  • Corticosteroids: These are often used as

Recommended Medications

  • Corticosteroids

💊 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 diseases affecting the peripheral nervous system (PNS) can be challenging to diagnose, as they often present with non-specific symptoms and can mimic other conditions. Here are some key points to consider when making a differential diagnosis:

  • Infections: Certain viral infections, such as West Nile virus, enterovirus, and poliovirus, can cause neurological symptoms that may resemble autoimmune PNS disorders [6].
  • Myasthenia gravis: This is an autoimmune disease that affects the neuromuscular junction, leading to muscle weakness and fatigue. It can be a differential diagnosis for autoimmune PNS disorders, particularly those affecting the peripheral nerves [6].
  • Multiple sclerosis: While primarily a central nervous system (CNS) disorder, multiple sclerosis can sometimes present with symptoms that overlap with autoimmune PNS diseases. The presence of anti-AQP-4 and anti-MOG antibodies can help differentiate between these conditions [8].

Other differential diagnoses to consider include:

  • Guillain-Barré syndrome: This is an autoimmune disorder that affects the peripheral nerves, leading to muscle weakness and paralysis. It can be a differential diagnosis for autoimmune PNS diseases, particularly those with rapid progression [10].
  • Inflammatory neuropathies: Conditions such as chronic inflammatory demyelinating polyneuropathy (CIDP) and vasculitis can also present with symptoms similar to autoimmune PNS disorders.

To determine the correct diagnosis, a careful clinical assessment, judicious laboratory testing, and electrodiagnostic studies or nerve biopsy may be necessary [9].

References:

[6] Aug 20, 2024 — Frequent differential diagnoses include infections (eg, West Nile virus, enterovirus, and poliovirus), myasthenia gravis, and other autoimmune ...

[8] The determination of anti-AQP-4 and anti-MOG antibodies enables early delimitation from multiple sclerosis, the most important differential diagnosis.

[9] by H AZHARY · 2010 · Cited by 277 — The diagnosis requires careful clinical assessment, judicious laboratory testing, and electrodiagnostic studies or nerve biopsy if the diagnosis ...

[10] by E Pascual-Goñi · 2024 · Cited by 2 — Autoimmune neuropathies are a heterogeneous group of immune-mediated disorders of the peripheral nerves. Guillain-Barré syndrome (GBS) and ...

Additional Differential Diagnoses

Additional Information

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