Summary and Overview
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune disorder causing progressive muscle weakness and sensory dysfunction in the limbs due to immune-mediated myelin damage. It often follows a relapsing or progressive course, with symptoms like numbness, tingling, pain, fatigue, and reduced reflexes. Early diagnosis and treatment are essential to prevent irreversible nerve damage and disability. Diagnosis involves clinical evaluation, electrodiagnostic studies, cerebrospinal fluid analysis, and adherence to EFNS/PNS criteria, though delays and misdiagnoses remain common.
Treatment includes intravenous immunoglobulin (IVIG), corticosteroids, and plasma exchange, benefiting about two-thirds of patients. Newer therapies like subcutaneous immunoglobulin (HYQVIA) and FcRn inhibitors such as efgartigimod, approved in 2024, show promise but require personalized management due to varied responses and risks during therapy changes. Care increasingly emphasizes individualized approaches coordinated by specialized teams to improve outcomes and quality of life.
Symptoms and Diagnosis
CIDP mainly presents with progressive muscle weakness, numbness, tingling, pain, fatigue, balance issues, and impaired walking. Symptoms can be relapsing or progressive, often resembling Guillain-Barré syndrome, leading to misdiagnosis. Delayed diagnosis increases the risk of refractory disease and permanent disability.
Diagnosis combines clinical assessment with nerve conduction studies detecting demyelination, CSF analysis showing elevated protein, and MRI findings. EFNS/PNS criteria guide diagnosis, with sensory criteria and red flags (e.g., pain, monoclonal gammopathy) improving accuracy. Biomarkers like serum neurofilament light chain are under investigation but not yet widely used clinically.
Treatment Options
Standard CIDP treatments are IVIG, corticosteroids, and plasma exchange, with about 10-15% of patients resistant to these therapies. HYQVIA, a subcutaneous immunoglobulin combined with hyaluronidase, is FDA- and EU-approved for maintenance therapy, demonstrating safety and efficacy. FcRn inhibitors such as efgartigimod reduce pathogenic IgG levels and were approved in 2024, but their effectiveness and safe transition from IVIG require further study due to risks of relapse and variable patient responses.
Treatment plans must be personalized, with ongoing research exploring new therapies to improve management.
Patient Care and Support
Patients with CIDP often need coordinated care due to complex health needs. Integrated care models improve communication among providers and ease healthcare navigation, benefiting patients and caregivers. Many patients rely on caregivers for around five hours daily, highlighting the importance of support systems. Treatment guidelines recommend therapies like IVIG and corticosteroids for moderate to severe cases, with resources available to assist patients in accessing treatments.
Healthcare workforce improvements and digital innovations are shaping future patient support and care delivery.
Cost and Economic Impact
CIDP imposes significant economic burdens, with higher direct and medication costs linked to greater disability and depressive symptoms. Indirect costs arise from caregiver support and reduced productivity. Treatments like HYQVIA have regulated pricing within the EU. Addressing these costs is vital for optimizing healthcare resource allocation and patient outcomes.
Patient Expectations and Prognosis
CIDP has a variable course, often requiring long-term monitoring and treatment adjustments. Symptoms affect limb strength, sensation, coordination, and daily functioning, with some patients achieving remission while others need continuous management. Functional impairment is assessed using standardized scales. New therapies like FcRn blockers offer hope but necessitate individualized treatment due to risks during therapy transitions.
Research and Future Directions
Research focuses on developing innovative CIDP treatments and improving patient outcomes. FcRn inhibitors such as efgartigimod show promise but highlight challenges in therapy switching and patient variability. Personalized treatment approaches remain essential as advances continue. Takeda’s ADVANCE clinical program supports ongoing investigation of these therapies.
The content is provided by Blake Sterling, Front Signals
