cidp treatment chronic

Treatment of dys-immune neuropathies.

Nobile-Orazio E.

Department of Neurological Sciences Dino Ferrari Center, University of Milan IRCCS Humanitas Clinical Institute, Via Manzoni 56, 20089 Rozzano, Milan, Italy. eduardo.nobile@unimi.it

Several therapies are currently used in dys-immune neuropathies including steroids,plasma exchange (PE), high-dose intravenous immunoglobulins(IVIg), and immuno-suppressive agents (IS). Even if there is substantial evidence that these treatments may improve the course of the neuropathy, their effectiveness is far from being complete and is sometime hampered by the occurrence of associated side effects. In Guillain-Barre syndrome (GBS),IVIg and PE are similarly effective in accelerating the recovery but there is still little evidence that they can reduce mortality or long-term disability. Recent reports on the association of intravenous methylprednisolone or interferon-beta (IFN-beta) to IVIg did not result in significant further improvement. In chronic inflammatory demyelinating polyradiculoneuropathy(CIDP) steroids, PE, and IVIG are initially similarly effective. The short-term effect of PE and IVIgand the side effects associated with the long-term use of steroids have prompted the use of several IS, interferon and,more recently, the anti-CD20 monoclonal-antibody Rituximab, but their efficacy has still to be proved in controlled studies. The recent identification of multifocal motor neuropathy(MMN) was shortly followed by the finding of an effective therapy. Almost 80% of patients respond toIVIg whose effect needs to be maintained with periodic infusions for long periods of time, and tends to decrease after several years. Also in this condition a number of immune modulating agents have been used to reduce the frequency or improve the effectiveness of IVIg,but their efficacy has not been sofar confirmed in randomized trials.Similar conclusions can be drawn for neuropathies associated with monoclonal gammopathies where only PE and IVIg have proved to be effective in controlled studies,while the promising initial results obtained with Rituximab in neuropathy associated IgM monoclonal gammopathy awaits confirmation from controlled trials.

Publication Types:

Review

PMID: 15834625 [PubMed - indexed for MEDLINE]

: Treatment of chronic inflammatory demyelinating polyradiculoneuropathy with methotrexate.

Fialho D, Chan YC, Allen DC, Reilly MM, Hughes RA.

Department of Molecular Neuroscience, Institute of Neurology, Queen Square, London WC1N 3BG, UK. doreen@doctors.org.uk.

We discovered many reports of other immunosuppressive drugs being used in adults with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) but none of methotrexate. As weekly low dose oral methotrexate is safe, effective, and well tolerated in other diseases, we treated 10 patients with otherwise treatment resistant CIDP. Seven showed improvement in strength by at least two points on the MRC sum score and three worsened. Only two showed an improvement in disability and both were also receiving corticosteroids. We discuss the difficulty of detecting an improvement in treatment resistant CIDP and propose methotrexate as a suitable agent for testing in a randomised trial.

PMID: 16543541 [PubMed - in process]

: Chronic inflammatory demyelinating polyneuropathy: a treatment protocol proposal.

Odaka M.

Department of Neurology, Dokkyo University School of Medicine, Kitakobayashi 880, Mibu, Shimotsuga, Tochigi 321-0293, Japan. m-odaka@dokkyomed.ac.jp

Guidelines for diagnostic criteria and treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) have been proposed by a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society, based on available evidence and expert consensus. These should prove practical for the clinical management of CIDP. Intravenous immunoglobulin followed by corticosteroids should be considered as the initial treatment, however no clear second drug of choice for patients who do not respond to the initial treatment is given. The author reports the long-term therapeutic efficacy of cyclosporin for patients with CIDP who did not show sustained improvement under steroid therapy. Cyclosporin should be tried for patients with intractable CIDP who require repeated intravenous immunoglobulin. An adequate initial dose of cyclosporin is 3 mg/kg/day, with plasma trough concentrations between 100 and 150 ng/ml. If patients respond to cyclosporin, remission can be maintained for 2 years, after which the dose can be slowly reduced over 1 year. Eventual withdrawal should be considered. This review proposes a treatment strategy that includes long-term maintenance therapy for CIDP based on published clinical trials and the author's clinical experience. Current concepts concerning the clinical spectrum of CIDP and diagnostic approaches are also considered.

PMID: 16533141 [PubMed - in process]

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