About Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Overview

CIDP is an autoimmune disorder in which an immune response is directed toward the myelin sheath surrounding peripheral nerves. While symptoms are variable, patients with CIDP generally suffer from chronic, relatively symmetrical motor weakness and sensory loss, developing over at least two months. CIDP can be progressive, relapsing-remitting or monophasic and can result in significant disability.1 CIDP is the most common peripheral autoimmune demyelinating neuropathy with a prevalence of 1.2-7.7 per 100,000 globally.2 At any one time, CIDP is thought to affect up to 40,000 people in the US.3 The disorder is most likely to develop in patients between the ages of 60 and 70, and is twice as common in males as in females.3 

Signs and Symptoms

Diagnosing CIDP can be challenging due to wide variations in clinical symptoms. Typical, or "classic" CIDP generally refers to cases in which both motor and sensory neurons are affected, however, only 50% of patients fall into this category.4

Most CIDP patients experience3:

> Muscle weakness
> Loss of deep tendon reflexes
> Poor balance
> Loss of sensation (maximal in hands and feet)

Diagnosis

Early recognition and diagnosis of CIDP are important, as patients are more likely to respond to treatment if it is begun early in the disease course.3  A diagnosis of CIDP requires a combination of clinical signs in addition to evidence of peripheral nerve demyelination.4 

Treatment

Treatment for CIDP includes immunomodulating, anti-inflammatory and immunosuppressive therapies.

Treatment goals usually include5:

> Symptom reduction
> Functional status improvement
> Maintenance of long-term remission

 


References1. Kerr J, et al. Is dosing of therapeutic immunoglobulins optimal? A review of a three-decade long debate in Europe. Front Immunol. 2014;5:629. 2. Bright RJ, Wilkinson J, Coventry BJ. Therapeutic options for chronic inflammatory demyelinating polyradiculoneuropathy: a systematic review. BMC Neurol. 2014;14:26. 3. Steinberg JS, Koski CL. Guillain-Barré syndrome, CIDP and variants. An overview for the layperson. 10th ed. https://www.gbs-cidp.org/wp-content/uploads/2012/01/OverviewENG.pdf. Accessed May 22, 2018. 4. Mathey EK, Park SB, Hughes RAC, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype. J Neurol Neurosurg Psychiatry. 2015;86:973-85. 5.Gorson KC. An update on the management of chronic inflammatory demyelinating polyneuropathy. Ther Adv Neurol Disord. 2012;5:359-73. 

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Important Safety Information

GAMMAKED™ [Immune Globulin Injection (Human) 10% Caprylate/Chromatography Purified] is an immune globulin injection that is approved to treat primary humoral immunodeficiency (PI) in patients 2 years of age and older, idiopathic thrombocytopenic purpura (ITP) in adults and children, and chronic inflammatory demyelinating polyneuropathy (CIDP) in adults.

Boxed Warning: Thrombosis, Renal Dysfunction and Acute Renal Failure

  • Thrombosis may occur with immune globulin products, including GAMMAKED. Risk factors may include: advanced age, prolonged immobilization, hypercoagulable conditions, history of venous or arterial thrombosis, use of estrogens, indwelling vascular catheters, hyperviscosity, and cardiovascular risk factors. Thrombosis may occur in the absence of known risk factors.
  • For patients at risk of thrombosis, administer GAMMAKED at the minimum dose and infusion rate practicable. Ensure adequate hydration in patients before administration. Monitor for signs and symptoms of thrombosis and assess blood viscosity in patients at risk for hyperviscosity.
  • Renal dysfunction, acute renal failure, osmotic nephrosis, and death may occur with immune globulin intravenous (IGIV) products in predisposed patients. Patients predisposed to renal dysfunction include those with any degree of pre-existing renal insufficiency, diabetes mellitus, age greater than 65, volume depletion, sepsis, paraproteinemia, or patients receiving known nephrotoxic drugs.
  • Renal dysfunction and acute renal failure occur more commonly in patients receiving IGIV products containing sucrose. GAMMAKED does not contain sucrose.
  • For patients at risk of renal dysfunction or failure, administer GAMMAKED at the minimum concentration available and the minimum infusion rate practicable.

GAMMAKED is contraindicated in patients who have had an anaphylactic or severe systemic reaction to human immunoglobulin. It is also contraindicated in IgA deficient patients with antibodies against IgA and history of hypersensitivity. Have epinephrine available immediately to treat any acute severe hypersensitivity reactions.

Hyperproteinemia, increased serum viscosity, and hyponatremia may occur in patients receiving IGIV therapy, including GAMMAKED.

Aseptic Meningitis Syndrome (AMS) may occur infrequently, especially with high doses or rapid infusion.

Hemolysis, either intravascular or due to enhanced red blood cell (RBC) sequestration, can develop subsequent to GAMMAKED treatment. Risk factors include high doses and non-O blood group. Closely monitor patients for hemolysis and hemolytic anemia, especially in patients with pre-existing anemia and/or cardiovascular or pulmonary compromise.

Noncardiogenic pulmonary edema may occur in patients following treatment with IGIV products, including GAMMAKED. Monitor patients for pulmonary adverse reactions (transfusion-related acute lung injury [TRALI]).

The high dose regimen (1g/kg x 1-2 days) is not recommended for individuals with expanded fluid volumes or where fluid volume may be a concern.

GAMMAKED is made from human plasma. Because this product is made from human plasma, it may carry a risk of transmitting infectious agents, e.g., viruses, the variant Creutzfeldt-Jakob disease (vCJD) agent and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent.

GAMMAKED is not approved for subcutaneous use in patients with ITP. Due to the potential risk of hematoma formation, GAMMAKED should not be administered subcutaneously in patients with ITP.

After infusion of IgG, the transitory rise of the various passively transferred antibodies in the patient's blood may yield positive serological testing results, with the potential for misleading interpretation.

In clinical studies, the most common adverse reactions with GAMMAKED (≥5% of subjects) were: (in PI intravenous) cough increased, rhinitis, pharyngitis, headache, asthma, nausea, fever, diarrhea, and sinusitis; (in PI subcutaneous) local infusion site reactions, fatigue, headache, upper respiratory tract infection, arthralgia, diarrhea, nausea, sinusitis, bronchitis, depression, allergic dermatitis, migraine, myalgia, viral infection, and pyrexia; (in ITP) headache, ecchymosis, vomiting, fever, nausea, rash, abdominal pain, back pain, and dyspepsia; (in CIDP) headache, pyrexia, hypertension, chills, rash, nausea, arthralgia, and asthenia.

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