Management of Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Abstract

This review briefly describes current concepts concerning the nosological status, pathogenesis and management of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). CIDP is an uncommon variable disorder of unknown but probably autoimmune aetiology. The commonest form of CIDP causes more or less symmetrical progressive or relapsing weakness affecting proximal and distal muscles. Against this background the review describes the short-term responses to corticosteroids, intravenous immunoglobulin (IVIg) and plasma exchange that have been confirmed in randomised trials. In the absence of better evidence about long-term efficacy, corticosteroids or IVIg are usually favoured because of convenience. Benefit following introduction of azathioprine, cyclophosphamide, cyclosporin, other immunosuppressive agents, and interferon-beta and -alpha has been reported but randomised trials are needed to confirm these benefits. In patients with pure motor CIDP and multifocal motor neuropathy, corticosteroids may cause worsening and IVIg is more likely to be effective. General measures to rehabilitate patients and manage symptoms, including foot drop, weak hands, fatigue and pain, are important.

Pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy

“…There is a regrettable lack of information from controlled trials about the efficacy of immunosuppressive drugs. Responses to several drugs, particularly cyclosporine and cyclophosphamide, have been reported in observational studies ( Mahattanakul et al, 1996 ; Good et al, 1998 ; Hughes, 2003 ) . We have seen improvements in strength with methotrexate in seven of 10 patients ( Fialho et al, 2006 ) .…”

“…In doing so, we have focused on the typical symmetrical sensory and motor core syndrome ( Hughes et al, 2005 ) and taken into account other recent reviews of the same topic ( Kieseier et al, 2004 ; Rezania et al, 2004 ) , presenting the evidence for each of the main proposals regarding etiology. Treatment of CIDP with immunomodulatory agents is only beneficial in two‐thirds of patients, and even in these the treatment response is often unsatisfactory ( Donofrio, 2003 ; Hughes, 2003 ) . A better understanding of the disease mechanisms should guide the design of better treatments.…”

Chronic inflammatory demyelinating polyneuropathy triggered by interferon‐α for chronic hepatitis B virus infection

“…Several reports have shown that administration of IFN-a is effective for patients with CIDP that was refractory to conventional therapies. 2 We speculate that IFN-a might trigger a pathological autoimmune reaction, such as molecular mimicry, in some immunologically-predisposed patients, while suppressing pathological autoimmune activation. 3 In the previous reports, CIDP associated with IFN-a occurred in patients with HCV infection, 1,4-7 except in a few cases with malignant melanoma.…”

Predicting treatment response in chronic, acquired demyelinating neuropathies

“…The effects of IVIg and PE treatment are usually seen early, but their effects are often short lived. Regular repeat treatment courses at intervals of between 2-12 weeks are usually needed to maintain improvement [15].…”