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Cyclosporin A in treatment of refractory patients with chronic inflammatory demyelinating polyradiculoneuropathy.
Matsuda M, Hoshi K, Gono T, Morita H, Ikeda S.
The Third Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano 390-8621, Japan. firstname.lastname@example.org
To investigate the therapeutic efficacy of cyclosporin A (CyA) in the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), a microemulsion form of this drug (Neoral) was orally given to seven patients with the disease who were unresponsive or resistant to conventional therapies. The daily dose of CyA was carefully controlled in order to keep the plasma trough concentration between 100 and 150 ng/ml. Within 1 month of initiation of CyA, all patients subjectively showed improvement of clinical symptoms, while both modified Rankin and INCAT disability scores were significantly decreased (p < 0.05) and grip strength was significantly increased (p < 0.05) 3 months after initiation compared with before. Total protein in the cerebrospinal fluid was significantly decreased 3 and 6 months after starting CyA (p < 0.05). Although the maximal motor nerve conduction velocity showed a significant improvement in the median nerve 1 to 1.5 years after commencement of CyA (p < 0.05), there were no significant changes in any other neurophysiological parameters. One patient with anti-sulphoglucuronyl paragloboside IgM antibodies gradually became resistant to CyA, but the rest have since been in good neurological condition without complications ascribable to this drug. These results suggest that oral CyA may be effective even for refractory cases with CIDP. CyA should be actively considered as a therapeutic option when patients with CIDP are resistant to conventional treatment.
PMID: 15450768 [PubMed - indexed for MEDLINE]
Chronic inflammatory demyelinating polyneuropathy: a treatment protocol proposal.
Department of Neurology, Dokkyo University School of Medicine, Kitakobayashi 880, Mibu, Shimotsuga, Tochigi 321-0293, Japan.
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]