Symptoms can present in typical and variant ways
- CIDP can be highly variable among patients in the early course of their disease1,2
- Between 31% and 48% of patients initially present with variant symptoms3
- Misdiagnosis of the variant type of CIDP is common. The revised EAN/PNS guidelines attempt to improve the specificity of diagnostic criteria for CIDP variants4
“The clinical and pathological manifestations may … differ between patients, explaining the number of disease variants according to the distribution of signs and symptoms.”
Not all patients have a typical CIDP presentation1,2
Typical CIDP Symptoms4
- Slow onset over ≥8 weeks
- May have initial acute presentation
- Chronic or stepwise progression
- Symmetrical symptoms
- Distal and proximal weakness
- Sensory dysfunction
- Absent or reduced tendon reflexes in all extremities
CIDP Variants4
- Distal CIDP
- Multifocal CIDP
- Focal CIDP
- Motor CIDP
- Sensory CIDP
Though similar in presentation, there are key differences between typical CIDP and DPN5,6†
Typical CIDP4
- Distal and proximal weakness
- Motor and sensory loss
- Reduced or absent reflexes
- Symptoms evolve over months
- Significant pain is less likely
DPN6
- Distal weakness mainly in the feet
- Sensory loss, with no motor loss observed
- Most have absent DTRs at ankles
- Symptoms evolve over years
- Significant pain is more likely
The pathophysiology of CIDP
Early detection is critical as the patient can experience secondary axonal loss, resulting in potentially irreversible nerve damage and permanent disability.2
Confirming CIDP
Electrodiagnostic testing on 3 limbs increases diagnostic accuracy of variant CIDP. Testing of 2 limbs was sufficient to diagnose 92% of patients with typical CIDP, but only 66% of patients with CIDP variants8
These test results could mean CIDP5,9
- Prolonged distal latency
- Slow conduction velocity
- Delayed or absent F waves
- Partial conduction block
- Abnormal temporal dispersion
- Prolonged distal CMAP duration
GAMUNEX-C provides proven neuroprotection from inflammation in CIDP
Severe hypersensitivity reactions may occur with IVIG products, including GAMUNEX-C. In case of hypersensitivity, discontinue GAMUNEX-C infusion immediately and institute appropriate treatment.
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Important Safety Information
GAMUNEX®-C (immune globulin injection [human], 10% caprylate/chromatography purified) is indicated for the treatment of primary humoral immunodeficiency disease (PIDD) in patients 2 years of age and older, idiopathic thrombocytopenic purpura (ITP) in adults and children, and chronic inflammatory demyelinating polyneuropathy (CIDP) in adults.
Thrombosis may occur with immune globulin products, including GAMUNEX-C. Risk factors may include: advanced age, prolonged immobilization, hypercoagulable conditions, history of venous or arterial thrombosis, use of estrogens, indwelling central vascular catheters, hyperviscosity, and cardiovascular risk factors. Thrombosis may occur in the absence of known risk factors. For patients at risk of thrombosis, administer GAMUNEX-C 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 (IVIG) products in predisposed patients. Patients predisposed to renal dysfunction include those with any degree of preexisting 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 IVIG products containing sucrose. GAMUNEX-C does not contain sucrose. For patients at risk of renal dysfunction or failure, administer GAMUNEX-C at the minimum concentration available and the minimum infusion rate practicable.
GAMUNEX-C is contraindicated in patients who have had an anaphylactic or severe systemic reaction to the administration of human immune globulin. It is contraindicated in IgA-deficient patients with antibodies against IgA and history of hypersensitivity.
Severe hypersensitivity reactions may occur with IVIG products, including GAMUNEX-C. In case of hypersensitivity, discontinue GAMUNEX-C infusion immediately and institute appropriate treatment.
Monitor renal function, including blood urea nitrogen (BUN), serum creatinine, and urine output in patients at risk of developing acute renal failure.
Hyperproteinemia, increased serum viscosity, and hyponatremia may occur in patients receiving IVIG treatment, including GAMUNEX-C.
There have been reports of aseptic meningitis, hemolytic anemia, and noncardiogenic pulmonary edema (transfusion-related acute lung injury [TRALI]) in patients administered with IVIG, including GAMUNEX-C.
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.
Because GAMUNEX-C is made from human blood, it may carry a risk of transmitting infectious agents, eg, viruses, the variant Creutzfeldt-Jakob disease (vCJD) agent, and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent.
Do not administer GAMUNEX-C subcutaneously in patients with ITP because of the risk of hematoma formation.
Periodic monitoring of renal function and urine output is particularly important in patients judged to be at increased risk of developing acute renal failure. Assess renal function, including measurement of BUN and serum creatinine, before the initial infusion of GAMUNEX-C and at appropriate intervals thereafter.
Consider baseline assessment of blood viscosity in patients at risk for hyperviscosity, including those with cryoglobulins, fasting chylomicronemia/markedly high triacylglycerols (triglycerides), or monoclonal gammopathies, because of the potentially increased risk of thrombosis.
If signs and/or symptoms of hemolysis are present after an infusion of GAMUNEX-C, perform appropriate laboratory testing for confirmation.
If TRALI is suspected, perform appropriate tests for the presence of antineutrophil antibodies and anti-HLA antibodies in both the product and patient's serum.
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 GAMUNEX-C were headache, pyrexia, hypertension, chills, rash, nausea, arthralgia, and asthenia (in CIDP); cough, rhinitis, pharyngitis, headache, asthma, nausea, fever, diarrhea, and sinusitis with intravenous use (in PIDD) and local infusion-site reactions, fatigue, headache, upper respiratory tract infection, arthralgia, diarrhea, nausea, sinusitis, bronchitis, depression, allergic dermatitis, migraine, myalgia, viral infection, and pyrexia with subcutaneous use (in PIDD); and headache, ecchymosis, vomiting, fever, nausea, rash, abdominal pain, back pain, and dyspepsia (in ITP).
The most serious adverse reactions in clinical studies were pulmonary embolism (PE) in 1 subject with a history of PE (in CIDP), an exacerbation of autoimmune pure red cell aplasia in 1 subject (in PIDD), and myocarditis in 1 subject that occurred 50 days post-study drug infusion and was not considered drug related (in ITP).
Please see accompanying full Prescribing Information for GAMUNEX-C.
Terms to know
IG, immune globulin; CIDP, chronic inflammatory demyelinating polyneuropathy; PIDD, primary immunodeficiency disease; ITP, idiopathic thrombocytopenic purpura; Sub Q, subcutaneous; IV, intravenous; ICE, 10% caprylate-chromatography purified immune globulin intravenous (IGIV-C) CIDP efficacy.
References
- Hughes R. Chronic inflammatory demyelinating polyradiculoneuropathy. J Clin lmmunol. 2010;30(suppl 1):S70-S73.
- Dalakas MC. Advances in the diagnosis, pathogenesis and treatment of CIDP. Nat Rev Neurol. 2011:7(9):507-517.
- Allen JA. The misdiagnosis of CIDP: A review. Neurol Ther. 2020:9(1):43-54.
- Van den Bergh PYK, van Doorn PA, Hadden RDM, et al. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force-second revision. Eur J Neurol. 2021;28(11):3556-3583.
- Lotan I, Hellman MA, Steiner I. Diagnostic criteria of chronic inflammatory demyelinating polyneuropathy in diabetes mellitus. Acta Neurol Scand. 2015;132(4)278-283.
- Llewelyn JG. The diabetic neuropathies: types, diagnosis and management. J Neurol Neurosurg Psychiatry. 2003;74(suppl 2):ii15-ii19.
- Köller H, Kieseier BC, Jander S, Hartung HP. Chronic inflammatory demyelinating polyneuropathy. N Engl J Med. 2005;352(13):1343-1356.
- Vo ML, Hanineva A, Chin RL, Carey BT, Latov N, Langsdorf JA. Reply: Comparison of 2-limb versus 3-limb electrodiagnostic studies in the evaluation of chronic inflammatory demyelinating polyneuropathy. Muscle Nerve. 2015;51(4):549-553.
- Joint Task Force of the EFNS and the PNS. European Federation of Neurological Societies/Peripheral Nerve Society Guideline on Management of Chronic Inflammatory Demyelinating Polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society – first revision. J Peripher Nerv Syst. 2010;15(3):1-9.