Welcome to the Rare Classroom, a new series from Patient Worthy. Rare Classroom is designed for the curious reader who wants to get informed on some of the rarest, most mysterious diseases and conditions. There are thousands of rare diseases out there, but only a very small number of them have viable treatments and regularly make the news. This series is an opportunity to learn the basics about some of the diseases that almost no one hears much about or that we otherwise haven’t been able to report on very often.
Eyes front and ears open. Class is now in session.
The disease that we will be learning about today is:
Chronic Inflammatory Demyelinating Polyneuropathy
What is Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)?
- CIDP is characterized by progressive weakness and impaired sensory function in the arms & legs caused by damage to the myelin sheath of the peripheral nerves caused by the immune system attacking and damaging the myelin sheath
- CIDP presents itself with symptoms including tingling or numbness, weakness of the arms and legs, loss of deep tendon reflexes, fatigue, and abnormal sensations
- The reported incidence rate of CIDP varies from 1.6 to 8.9 per 100,000 people
- Approximately 1.6 per 100,000 people are newly diagnosed with CIDP per year
- There is a higher prevalence of CIDP in men than women, affecting males around twice as often
- The average age of onset of around 50 years old, but the disease can occur at any point in a person’s life
- Diagnosis is based on the symptoms a patient is showing, usually including numbness, tingling and pain, loss of reflexes, and weakness
How Do You Get It?
- CIDP has not been found to be hereditary and is considered an acquired disorder.
- Men are at greater risk
- Some cases have reported that after being treated with tumor necrosis factor-alpha inhibitors
- “Currently, neither the target antigen not the cell population responsible for the pathogenesis of CIDP have been identified…… More research is needed to establish the target of the T-cell response and whether other cell populations, like NK and T-cells are relevant to the pathogenesis of CIDP”
– Mahdi-Rogers, Rajabally
What Are The Symptoms?
- Physical Symptoms
- Loss of reflex functions
- Weakness around the hip, shoulder, hands, and feet, in both proximal and distal muscles
- Muscle loss
- Blurred Vision
- Neurological Symptoms
- Loss of sensations (numbness)
- Abnormal Sensations in extremities (tingling and pricking sensations)
- Abnormal sensation of pain and burning
How Is It Treated?
- The goal of treatment is to remove harmful antibodies from the blood, add unaffected antibodies into blood plasma, reduce inflammation, and relieve symptoms
- The main first line treatments for CIDP are Corticosteroids, High Dose Intravenous Immune Globulins (IVIG), and Plasma Exchange (PE), or Plasmapheresis (PLEX)
- Drug treatment is administered intravenously and orally, and usually requires long-term care of patients and ongoing treatments
- Healthcare team includes therapists (if undergoing physical therapy), rehabilitation specialists, and neurologists
- Factors impacting treatment/prognosis include similarity to other autoimmune and neuropathic disorders, as well as side effects that may coincide with standard treatments
- Approved Treatments:
- GAMUNEX®-C (immune globulin injection [human], 10% caprylate/chromatography purified) is approved to treat primary humoral immunodeficiency disease (PIDD) in patients 2 years of age and older. If you have PIDD, you may take GAMUNEX-C under the skin (subcutaneously) or in a vein (intravenously). GAMUNEX-C is also approved to treat idiopathic thrombocytopenic purpura (ITP) and chronic inflammatory demyelinating polyneuropathy (CIDP). If you have ITP or CIDP, you may only take GAMUNEX-C intravenously.
- Corticosteroids (Prednisone, Prednisolone) are used as an initial treatment. Side effects can be a barrier to long-term use
- Intravenous Immune Globulins (IVIG) was the only approved drug approved for treatment of CIDP until recently
- Subcutaneous Immune Globulin injections are currently in clinical trial as an alternative to intravenous immune globulin treatment
- Plasma Exchange (PE) or Plasmapheresis (PLEX) can be used to treat CIDP, this is where a patient’s blood is removed, filtered for harmful antibodies, and returned to the blood stream.
- Physical and Occupational Therapy may improve muscle strength, function, and aptitude, and diminish the shrinkage of muscles and tendons in the body.