A review of the medical management of chronic inflammatory demyelinating polyradiculoneuropathy

Abstract

Chronic idiopathic demyelinating polyradiculoneuropathy (CIDP) is a rare condition, but merits consideration due to its disabling consequences for patients and the growing existence of efficacious therapies during the last few decades. The first step is to characterise this neuropathy among the chronic dysimmune polyneuropathies, according to clinical, electrophysiologicalal and sometimes pathologicalal and immunochemical criteria. Typical CIDP is currently defined by criteria which have progressively improved since the first attempt made by an Ad Hoc Subcommittee of the American Academy of Neurology in 1991. However, CIDP may be associated with several concurrent diseases, and other chronic demyelinating polyneuropathies may be considered as either subtypes of CIDP, such as sensory CIDP and multifocal acquired sensory and motor neuropathy, or frontiers of CIDP, such as multi-focal motor neuropathy and polyneuropathy associated with monoclonal gammopathy. These considerations are helpful in the choice of treatments, as the response to immunomodulatory agents is different according to the type of the dysimmune neuropathy. CIDP is considered to be an immune-mediated disorder and may respond dramatically to numerous short-term therapies, such as corticosteroids, plasma exchanges, or intravenous immunoglobulin. The aim of this review is both to summarise the main results of the published open and randomised controlled trials for CIDP, and to provide some information about randomised controlled trials currently in progress. The objectives of the current and future trials are firstly, to choose the best regimen for short-term treatments, and secondly, to test new immunosuppressants in long-term therapy, if the neurological condition requires it.