From the Guidelines
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a rare autoimmune disorder characterized by progressive weakness and loss of sensory function in the legs and arms, resulting from inflammation and demyelination of the peripheral nerves. The exact definition and treatment of CIDP can be complex, but recent studies have shed some light on its management. According to a large observational study, patients with CIDP may derive symptomatic benefit from a dosing regimen of IVIg either alone or in conjunction with anti-TNF monoclonal antibody therapy 1. Some key points to consider about CIDP include:
- It is an autoimmune disorder that affects the peripheral nerves
- Symptoms can include progressive weakness, loss of sensory function, and pain in the legs and arms
- Treatment often involves immunomodulatory therapies such as IVIg and anti-TNF monoclonal antibody therapy
- The goal of treatment is to alleviate symptoms and improve quality of life, as there is currently no cure for CIDP
- Studies have shown that IVIg therapy can be effective in reducing symptoms and improving outcomes in patients with CIDP, with one study finding that 75% of patients derived symptomatic benefit from IVIg therapy either alone or in conjunction with anti-TNF monoclonal antibody therapy 1. In terms of management, the use of IVIg therapy is a recommended treatment option for CIDP, particularly for patients who have not responded to other treatments. Overall, CIDP is a complex and debilitating condition that requires careful management and treatment to alleviate symptoms and improve quality of life.
From the Research
Definition and Characteristics of CIDP
- Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is an acquired immune-mediated polyradiculoneuropathy that leads to disability via inflammatory demyelination of peripheral nerves 2.
- CIDP is characterized by neurological symptoms and signs of progressive weakness, paresthesias, and sensory dysfunction, with other symptoms including reduced or absent tendon reflexes, cranial nerve involvement, autonomic symptoms, ataxia, and neuropathic pain 3.
- The onset of CIDP is generally insidious and can take up to 8 weeks with a relapsing-recovery pattern, and it generally affects older individuals with a male predominance 3.
Diagnosis and Treatment of CIDP
- The diagnosis of CIDP is based on the clinical presentation and electrophysiology demonstrating demyelination in the peripheral nerves 4.
- CIDP can be successfully treated with immunoglobulin, glucocorticoids, or plasma exchange, and subcutaneous administration of immunoglobulin provides a new option for patients with CIDP that has the potential to increase independence and improve tolerability 5, 4.
- Treatment options also include corticosteroids, intravenous or subcutaneous immunoglobulin, or plasma exchange as first-line therapies, with immunosuppressants or rituximab used in special cases 2, 3.
- About 30% of patients will remit, and maintenance treatment will no longer be necessary 4.
Challenges in CIDP Management
- Despite various established therapies, the treatment of CIDP remains challenging due to its heterogeneous disease course and the lack of surrogate parameters to predict the risk of clinical deterioration 2.
- CIDP is difficult to diagnose, but early diagnosis can be crucial to prevent permanent nerve damage 5.
- The disease can have a significant impact on daily activity, resulting in impaired daily activity due to proximal and distal weakness and sensory disturbances 4.