What Is CIDP?
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare, debilitating, and degenerative neurologic illness characterized by progressive weakness due to peripheral nerve damage.
The exact cause is unknown but it is believed to be primarily autoimmune or inflammatory with no specific predisposing risk factors clearly identified.
Inflammation of nerve roots and peripheral nerves results in myelin sheath destruction resulting in progressive weakness and reduced functioning of extremities due to nerve damage.
Early recognition and proper treatment are critical in helping patients avoid a significant amount of disability.
Men are affected about twice as often as women and the condition increases in incidence with age. The gradual onset of CIDP can delay diagnosis by several months or even years, resulting in significant nerve damage that may limit and delay response to therapy.
Most individuals will require long-term treatment and if left untreated, an estimated 30% of patients would eventually become wheelchair dependent.
Study results have shown that patients with CIDP have 7.5 times higher mean healthcare costs when compared to those without CIDP.
What is CIDP?
CIDP is seen to occur more commonly in males and those of advanced age. Although there is currently no cure, there are treatment options available to slow down the progression of symptoms.
When this happens, nerve damage outside the brain ensues, followed by demyelinating polyneuropathy symptoms including:
- Weakness and tingling in the arms and legs
- loss of sensation and reflexes,
- eventually, paralysis if untreated.
Although there is no cure for this illness, early diagnosis and treatment can help manage symptoms and prevent irreversible nerve damage.
Eventually, when the myelin is damaged, nerve fibers will not be able to conduct electrical impulses to stimulate muscle contraction and transmit sensory information throughout the nervous system.
It may take some time for the first symptoms of inflammatory demyelinating polyneuropathy to be experienced. It is also important to understand that symptoms are not the same for everyone. Below are CIDP symptoms Mayo Clinic and Ameripharma have stated:
- Unexplainable numbness
- Tingling in the arms and legs
- Weakness in the limbs
- Loss of balance
- Loss of sensation
- Reduced reflexes
Aside from the symptoms stated above, if CIDP neuropathy is untreated, paralysis may occur. Since autoimmune Chronic Inflammatory Demyelinating Polyneuropathy cannot be cured, early diagnosis and treatment can help in preventing paralysis and irreversible nerve damage.
If you are experiencing any of the symptoms above, speak to a doctor to discuss the possibility of having the disease.
CIDP Vs. GBS
This condition is extremely rare with approximately 5-7 out of every 100,000 people diagnosed with the disease, according to RareDiseases.org. Moreover, this health condition is often confused with Guillain-Barre syndrome (GBS) because of the similarity of symptoms. Both are autoimmune diseases that affect the Myelin (an insulating layer or sheath that forms around nerves).
Rate of Progression
However, in Guillain-Barre syndrome (GBS), with a subtype referred to as acute inflammatory demyelinating polyneuropathy (Landry’s Ascending Paralysis), symptoms progress rapidly while in CIDP neurology, symptoms take longer to manifest. Therefore, it can be said that demyelinating polyradiculoneuropathy autoimmune disease cannot be diagnosed until the symptoms persist and worsen over a period of 8 weeks or longer.
Severity of Damage to Nerves
CIDP is related to t inflammatory demyelinating polyneuritis while in Guillain-Barre syndrome (AIDP), we talk about axonal and demyelinating polyneuropathy. Axonal CIDP means the nerves are directly damaged by the disease.
Evaluation, Testing, and Diagnosis
There is a differential diagnosis between the two conditions. Generally, diagnosing this illness is based on the symptoms of the patient which will be learned during the evaluation of the doctor. Next will be a physical exam. This, then, will be followed by tests such as the following:
- Nerve Conduction and EMG (usually showing a chronic polyneuropathy) – during this test, the nerve is stimulated with mild electrical impulses, usually with surface electrode patches attached to the skin.
- Spinal Fluid Analysis (usually showing elevated protein with normal cell count) – during this test, a needle is inserted into the lower part of the spine to obtain a small sample of the liquid surrounding your spinal cord.
- MRI – in this procedure, powerful magnetic fields and radio waves are being used to take pictures of the spinal cord and nerve bundles. This procedure can help in diagnosing by detecting damage to the nerves.
- Blood and Urine Tests (to rule out other disorders that may cause neuropathy and to look for unusual proteins)
Aside from the tests above, there is also a rare test that is being done and that is called nerve biopsy to look at microscopic changes in the nerve.
After medical diagnosis, treatment will follow. This, then, will be followed by a chronic inflammatory demyelinating polyneuropathy prognosis which speaks about the chance of recurrence or recovery of a patient from the condition of inflammatory demyelinating polyneuropathy.
According to a study, 26% of the patients with polyneuropathy who were treated using infusions were able to get a complete disappearance of all their demyelinating polyneuropathy symptoms.
Moreover, another 26% of the patients with refractory CIDP were able to walk with continuous immune treatments while 34% were able to walk without additional ones.
Only 14% of the total patients in the long-term prognosis study still had a severe disability and this is caused by different prognostic factors.
Aside from the age and sex of a patient, there are different prognostic factors that affect the treatment of a patient such as the following:
- Mode of onset
- Response of the patient to the medicine steroid treatment
- Motor electrodiagnostic feature
In the same study, it was reported that there is an 89.5% chance for patients to have all their symptoms go away or their health condition to improve as long as their response to the medication is great.
The Life Expectancy of CIDP Patients
Although chronic inflammatory CIDP is not fatal, and the life expectancy of a patient is comparable to someone who does not have the neurological disease, a patient’s quality of life can be significantly impacted.
The longer the illness goes untreated, the more nerve damage can permanently limit sensory and motor functions. If severe nerve damage sets in, patients may require assistance with daily living activities due to a lack of mobility.
If you are experiencing unexplainable numbness or a loss of reflexes in your limbs for a prolonged time, you may want to discuss the possibility of having the disease with your doctor.
The sooner you can identify the disease and begin treatment, the higher the chance you can reverse symptoms and prevent further damage to your nerves.
Aside from acute inflammatory neuropathy, there are a number of variants or types of Chronic Inflammatory Demyelinating Polyneuropathy. Below is a list of the variants of this health condition:
Symmetrical motor and sensory progressive neuropathy affecting proximal and distal muscles with loss of deep tendon reflexes
Multifocal Motor Neuropathy
This is a pure motor inflammatory demyelinating disorder in which there is asymmetric weakness in the distribution of individual nerves that can be confirmed by diagnostic nerve conduction results
This is a sensory-motor disorder in which there is sensory loss and weakness in the distribution of individual nerves. Diagnostic nerve conduction studies confirm the focal nerve involvement.
A condition where there is a sensory loss, pain, and poor balance with abnormal gait or walking. There is no weakness but frequently motor nerve conduction studies are abnormal in addition to sensory conduction studies.
A condition where there is weakness and loss of reflexes without sensory loss.
Early Diagnosis And Treatment
Although there is no cure for this illness, early diagnosis and treatment can help manage symptoms, prevent paralysis and irreversible nerve damage.
Patients with CIDP report weakness in the hips and legs, loss of balance, burning pain, and numbness of the hands or feet as the most bothersome symptoms.
Treatment goals are to alleviate symptoms, improve functionality, and achieve long-term remission.
Current treatment options include:
- Immunosuppressive Agents Intravenous
- Subcutaneous Immunoglobulin
- Plasma Exchange
Prognosis is good with early and appropriate treatment.
CIDP is one of several neuropathies that share demyelination and immune mediation in common and could be misdiagnosed with other conditions. The clinical features that distinguish CIDP from other peripheral neuropathies are the prominence of muscle weakness and the early involvement of upper extremity and proximal muscles as well as distal muscles.
While the initial suspicion for CIDP is clinical, the diagnosis is confirmed by the evidence of peripheral nerve demyelination by electrodiagnostic findings, by nerve biopsy, and exclusion of other disorders that may cause or mimic CIDP.
Patients with atypical features or diagnostic uncertainty should be referred to a neuromuscular specialist with expertise in the diagnosis of peripheral nerve disorders.
The Following Criteria Support The Diagnosis Of CIDP:
- Progression over at least two months
- Weakness more than sensory symptoms
- Symmetric involvement of arms and legs
- Proximal muscles involved along with distal muscles
- Widespread reduction or loss of deep tendon reflexes
- Increased cerebrospinal fluid (CSF) protein without pleocytosis
- Nerve conduction evidence of a demyelinating neuropathy
- Nerve biopsy evidence of segmental demyelination with or without inflammation
- Gait ataxia secondary to large fiber sensory loss
Nerve conduction studies of both sensory and motor nerves should be performed in all patients with suspected CIDP and is a critical component of the evaluation
There are no laboratory test findings that specifically point to CIDP. The purpose of testing is to look for disorders that are either associated with or mimic CIDP.
Laboratory evaluation in patients with suspected CIDP should include the following:
- Fasting Serum Glucose
- Glycated Hemoglobin (Hba1c)
- Serum Calcium And Creatinine
- Complete Blood Count
- Liver Function Tests
- Thyroid Function Studies
- Serum Protein Electrophoresis (Spep) And Immunofixation
- Serum Free Light Chain (Flc) Assay, Or 24-hour Urine Protein Electrophoresis (Upep) And Immunofixation
Cerebrospinal fluid (CSF) analysis is recommended in most patients with suspected CIDP and particularly for patients in whom the clinical and electrodiagnostic findings are inconclusive
Most patients experience a progressive course, but roughly one-third have a relapsing/remitting or monophasic pattern. Improving symptoms, restoring functionality, and achieving long-term remission are the goals of treatment. Patients may need treatment indefinitely.
When immunoglobulins (IGs) are used, the results of research suggest that 75% of patients will improve.
About one-third of patients experience remission or cure, and an additional 44% will have stable active disease.
Early administration of treatment to suppress immune response is important in CIDP. The goal is to stop the immune attack against the myelin sheath of peripheral nerves. This can improve symptoms and function and can prevent or minimize long-term disability.
Treatment Options for CIDP Patients
Inflammatory neuropathy is an autoimmune disease. Therefore, what will be addressed are the symptoms and treatment that will be used must be able to suppress the immune response that is causing the attack of the myelin.
Since this condition is a type of autoimmune disease, the prescribed treatments often work to suppress the immune response that is causing the attack of the myelin.
CIDP generally responds to immunosuppressive or immunomodulatory treatment with glucocorticoids, intravenous immune globulin, or plasma exchange.
Early administration of effective treatment is important in CIDP. The goal is to stop the immune attack against the myelin sheath of peripheral nerves so that secondary axonal degeneration is minimized. This can improve symptoms and function and can prevent or minimize long-term disability.
Below are the possible treatments for Chronic Inflammatory Polyneuropathy and Neuropathy:
- Corticosteroids – These are similar to anti-inflammatory hormones made by the body and are used to suppress the body’s immune system. Additionally, these medications can improve the strength of a patient. Moreover, these are inexpensive drugs but their side effects can limit the patient’s use of them.
- Intravenous Immunoglobulin such as Gammagard, Privigen, or ArgenX – This is the new treatment for this illness and the only FDA-approved for the treatment of disease. This medication therapy is used to infuse antibodies into the blood.
- Plasmapheresis – This is a plasma exchange process that is used to remove harmful antibodies contained in the plasma.
Aside from these medications, immunosuppressive medicines, doctors may also recommend physical therapy to help improve mobility. Treatment can help increase physical strength, balance, and coordination to provide an improved quality of life.
In one clinical study, it was reported that the drug Rozanolixizumab is proven to be safe and effective for the elderly and pediatrics. Together with the medical treatments, there will be a chance for the patient to have increased physical strength, balance, and coordination.
This, then, may lead them to have an improved quality of life.
The best treatment for this condition is IVIg infusion therapy. Immune globulin infusion is an IV-based treatment designed to help patients with weakened immune systems. Infusion treatment delivers antibodies directly and regularly to patients through IV to fight off infections and inflammation.
Moreover, this product may produce mild to more severe side effects.
This medication also comes with side effects and it is important to take measures in order to minimize these side effects. Examples of these measures are the following:
- Stay hydrated
- Take the pre-medications adherently
- Listening to what your body is trying to tell you
Unexpected side effects may occur during the course of treatment. With the exception of anaphylaxis or allergic reaction, most of the side effects are common and sometimes, moderate or severe.
Moreover, whether moderate or severe, whatever side effects you are experiencing, always share them with your medical doctor. Below are the common side effects of this product:
- aching muscles
- stomach pains
- Hair Loss
- Weight gain
Moderate side effects of this product affect a patient’s daily activities such as going to work, sleeping well, and appetite. Aside from these, below are the moderate side effects of this medication:
- Pain in the lower back
Another moderate side effect of this medication is a headache but it is only considered moderate when it lasts for more than 24 hours and on a scale of 1-10, the pain can be described at a rate of 6-8.
If this headache does not go away, it can lead to a serious condition called aseptic meningitis, which occurs when there is an irritation of the meninges in the brain and spinal cord. This is a rare side effect. Moving on, below are the other rare or long-term side effects of the medication:
- aseptic meningitis (inflammation of the brain)
- liver problems
- kidney failure
- hemolytic anemia (a condition that destroys red blood cells)
Infusion treatments have been considered a remarkably good safety record for long-term administration. However, in some cases, it has been reported that this product causes adverse effects such as the following:
- Myalgia (fever)
- Blood pressure changes
- Anaphylactic reactions
Aside from the adverse effects listed above, there are also diseases associated with the long-term treatment of immunoglobulin infusions:
- Renal Dysfunction – This disease is more common in patients who are over 65 years old, who have pre-existing conditions such as hypertension and diabetes. Therefore, any changes in urination, including color changes (dark-colored urine) must be notified to a physician.
- Blood Clots – Thrombotic events or clot formation have been reported with the use of this product. Although this is very rare to occur, all patients must be aware of it. Blood clots can happen in elderly patients with a history of thrombotic events, diabetes, and heart problems. Usually, a clot is formed a few hours after the infusion. Seek emergency care or call 911 for immediate assistance.
- Anaphylaxis – This is the most serious adverse effect that is likely to occur during treatment and it appears within the first 15-30 minutes of infusion. This condition is characterized by difficulty of breathing, changes in the gastrointestinal system, cardiovascular changes, and skin changes such as rashes or hives. If anaphylaxis is suspected, immediately call a doctor or 911.
Moreover, this product may affect how vaccines work. Before getting a vaccine during this time of the pandemic, tell the medical professional about your condition in order to avoid immune globulin and covid vaccine interaction.
Pre-medications such as aspirin and Benadryl are often administered before the procedure to address these known side effects.
Additionally, administering the immune globulin at a slower rate may help prevent or lessen the severity of these side effects. Experienced infusion centers and nurses will often take precautions to prevent or help minimize any side effects before beginning treatment.
At Ameripharma, we have experienced and caring nurses that work hard to ensure your comfort and safety. A number of patients don’t realize IVIg Infusion can be done from the comfort of your home and may be covered by your medical insurance.
If you experience any of the side effects listed above, you may be given medications to alleviate the symptoms after your treatment session.
- Acetaminophen or ibuprofen is often given to relieve headaches and fever symptoms including high body temperatures and body aches.
- Anti-nausea medication can be given to help with nausea and vomiting.
- Benadryl is also commonly administered in the presence of anaphylactic symptoms including hives, rashes, and shortness of breath.
According to the Immune Deficiency Foundation, half of the infused immune globulin is metabolized within a three to four week period. Since IVIg is meant to infuse larger amounts of Ig, patients can normally go three to four weeks between treatments.
SCIg, on the other hand, delivers smaller amounts of Ig and takes longer to be absorbed which often requires more frequent infusion intervals ranging from daily to weekly.
Moreover, a patient with Chronic Inflammatory Demyelinating Polyneuropathy must also have a proper diet and healthy lifestyle. This includes avoiding drinking alcohol. In one clinical study, it was reported that CIDP and alcohol are related. When a patient with this condition consumes alcohol, the nerves become more damaged and abnormal because of nutritional deficiencies.
What to Expect with CIDP?
Soon after being treated with IVIg, daily activities such as climbing stairs and walking without assistance may be possible. Without treatment, however, symptoms of this disease may lead to nerve damage including paralysis and disability. But with treatment, the quality of life may improve and it may be possible for the disease to be in remission for some time.
Is CIDP Fatal?
No, this condition is not fatal. What only happens to a patient with this condition is that the quality of life of the patient is affected severely. The Longer the disease goes untreated, the more damage to the sensory and motor functions of the patient occurs.
Is demyelinating polyneuropathy life-threatening?
Idiopathic demyelinating polyneuropathy will only become life-threatening when it goes untreated. It is not fatal but it may cause a patient to be disabled for life.
What is the difference between MS and Chronic Inflammatory Polyneuropathy?
In Chronic Inflammatory Demyelinating Polyradiculoneuropathy vs MS, both multiple sclerosis (MS) and demyelinating polyneuropathy involve damage to the sheath that surrounds nerves, called myelin. But multiple sclerosis is a disease affecting the central nervous system, which includes the brain and spinal cord while the other condition doesn’t affect these areas of the body.
Does exercise help CIDP?
Exercise does help a patient with inflammatory peripheral neuropathy improve his or her condition. However, only an appropriate exercise is advisable in order to properly improve the strength and endurance of a patient.
Can you live a normal life with CIDP?
It is possible for patients with inflammatory demyelinating polyradiopathy to live a normal life just like others as long as the patient is getting immunoglobulin therapy and treatment.
How fast does CIDP progress?
Peripheral neuropathy is a condition that is slowly progressing unlike acute demyelinating polyradiculoneuropathy or Guillain-Barré syndrome. At around 8 weeks after nerve damage, symptoms start to worsen.
Can you fully recover from CIDP?
There are different classes of this condition such as progressive, recurrent, and monophasic. So, if you are wondering if a patient can still recover from this disease, the answer is yes. A patient may have a monophasic class and when a patient gets this class, he or she will only have to deal with the condition for around 1-3 years. After this, then, a patient will be free from this autoimmune illness.
Can CIDP turn into MS?
Even if this condition goes untreated, it will not turn into MS. However, you would not want to wait for your condition to worsen. It is advisable to treat it as soon as possible in order to avoid unwanted events.
Is CIDP hereditary?
No, this condition is not hereditary and it is not contagious as well.
How long does it take to recover from CIDP?
The CIDP recovery time for atypical or other variants of the disease depends on a lot of factors including the individual factors of a patient such as his or her health condition and response to the therapy. As long as a patient is able to respond positively to the therapy, recovery will be in no time.
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Dr. Christine Leduc, Pharm. D. was born and raised in Irvine, CA. She attended college at Midwestern University, where she graduated cum laude. The most rewarding part of her job is suggesting lifestyle changes, educating patients on how their medication works, and precepting future Pharmacists. She is currently precepting students from Marshall B. Ketchum University, University of Kansas, and Midwestern University. In her free time, she enjoys traveling, baking, and gardening.