Initial and Long-term Management of CIDP

Carol L. Koski, M.D., Principal Author

The EFNS/PNS joint task force issued "good practice points" for initial management and long-term management of cchronic inflammatory demyelinating polyneuropathy (CIDP ).

Initial Management of CIDP

1. Patients with very mild CIDP symptoms (e.g., those that do not or only slightly interfere with daily living activities) may be monitored without starting initial therapy.
2. Urgent treatment with corticosteroids or IVIG therapy should be considered for patients with moderate or severe disability (e.g., when hospitalization is required or ambulation is severely impaired).
3. Common initial doses of corticosteroids are prednisolone or prednisone 1 mg/kg or 60 mg daily, but a wide variation exists in practice.
4. The usual first dose of IVIG is 2.0 g/kg, given either as 0.4 g/kg on five consecutive days or as 1.0 g/kg each day for two days. The slower regimen would presumably limit infusion-related side effects particularly in older people. Contraindications to corticosteroids will influence the choice toward IVIG and vice versa. For pure motor CIDP, IVIG treatment should be the first choice, and if corticosteroids are used, patients should be monitored closely for deterioration.

Long-Term Management of CIDP

Since none of the random controlled trials systematically assesses long-term management of CIDP, no evidenced based guidelines could be given by the EFNS/PNS joint task force. Each patient requires assessment on an individual basis and recurrence of disease activity can lead to further nerve damage and disability.

For CIDP patients receiving corticosteroid therapy

A course of up to 12 weeks on their starting dose should be considered before deciding whether there is no treatment response for CIDP patients. If a response is seen, tapering the dose to the lowest effective maintenance dose every other day should be considered.

For CIDP patients receiving IVIG therapy

1. For patients starting on initial IVIG therapy, observation to discover occurrence and duration of any response to the first course should be considered before embarking on further IVIG treatment.
2. For patients who respond and then have condition worsen, further and ultimately repeated doses should be considered. Repeated doses should be given over one to two days. The amount given per course should be titrated according to individual response. Repeated courses of IVIG therapy may be needed every 2-6 weeks.
3. If a CIDP patient becomes stable on a regimen of intermittent IVIG therapy, the dose per course should be reduced before the frequency of administration is lowered.
4. If frequent high-dose IVIG is needed, the addition of either corticosteroids or an immunosuppressive agent should be considered.

Approximately 15% of patients fail to respond satisfactorily to any of these treatments. Some probably do not appear to respond because of secondary axonal degeneration.

Other CIDP Treatment Issues

1. Little evidence exists concerning the treatment of symptoms for CIDP, such as pain and fatigue.
2. However, fatigue is a major symptom in patients with immune-mediated polyneuropathies and may persist for years after apparent recovery. The Fatigue Severity Scale (FFS) is an appropriate tool for assessing fatigue in patients with CIDP.
3. There is limited research into the value of exercise and physiotherapy. No research has been done about advising the patient concerning immunizations.
4. International and national support groups offer information and support for patients.
5. Clinicians should consider recommending these organizations: GBS/CIDP Foundation International or GBS Support Group.

More Information on CIDP Treatment Issues

• See Treatment Issues