The prevalence of inflammatory neuropathies, immune-mediated disorders of the peripheral nerves, is higher than previously believed, and their correct management is important, experts reported at the World Congress of Neurology in Vienna. Researchers discussed the potential role of newer disease modifying agents in inflammatory neuropathies that have already proven effective in the therapy of Multiple Sclerosis or rheumatologic diseases.
Vienna, 23 September 2013 – “The scientific programme of this event reflects the enormous diversity and complexity of modern neurology”, Prof Richard Hughes (London), President of the European Federation of Neurological Societies (EFNS), said at the World Congress of Neurology in Vienna. More than 8,000 experts from all over the world are meeting in the Austrian capital to discuss latest developments in their field. Not only those neurological conditions with particularly high prevalence such as stroke or dementia are high on the agenda of this meeting, but also diseases that are less frequent, but cause a considerable burden and significant disability. Among those are inflammatory neuropathies such as Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal neuropathy (MMN), immune-mediated disorders of the peripheral nerves.
“Although the annual incidence of GBS remains steady at about 1 per 100,000, recent research suggests that the prevalence of CIDP and related disorders is far greater than previously thought, at about 10 per 100,000”, said Prof Hughes. “Collectively these inflammatory neuropathies are not uncommon and their detection, diagnosis and correct management are important.”
The past years have brought important advances in the management of these conditions. “Although there are now established treatments of proven benefit for inflammatory neuropathies consisting of intravenous immunoglobulins (IVIg), corticosteroids and plasma exchanges, there are patients who fail to respond to these therapies. Also, although many people with conditions like CIDP are treated, possibly not all of them receive optimal therapy”, Prof Hughes reported. “Currently the potential role of newer disease modifying agents effective in the therapy of multiple sclerosis or rheumatologic disease is being studied for inflammatory neuropathies.”
These research efforts are reflected in the programme of the Congress. Among others, a range of studies on the therapy of CIDP are being presented, a chronic sensomotor neuropathy causing significant disability.
Prof Hughes is leading member of a study group involving eight European centres who will update WCN delegates on the FORCIDP trial. This study aims at evaluating whether or not the oral drug fingolimod, which is already used in MS therapy, can delay disability progression in CIDP (Hughes et al). „The immunomodulating substance fingolimod completely prevented paraparesis, significantly decreased T-/B-cell, macrophage infiltration and demyelination of sciatic nerves in experimental autoimmune neuritis,” said Prof Hughes. “We now want to evaluate the efficacy of this substance in delaying disability progression as well as the drug’s safety and tolerability compared with placebo in CIDP patients.” FORCIDP is a double-blind, randomised, multi-centre, placebo-controlled, parallel-group study in adult patients with CIDP and history of disease activity upon discontinuation of CIDP therapy. “The primary outcome will be the time-to-first disability progression,” Prof Hughes explained.
The drug is not the only new treatment for CIDP discussed at the WCN, although some approaches seem less encouraging. A Canadian team (Garneau et al) presents data on the treatment of patients with myasthenia gravis, dermatomyositis, neuromyelitis optica, NMDA, encephalitis, vasculitis, and CIDP using the monoclonal antibody rituximab, which is established in oncology and rheumatology. Prof Hughes said the study concludes that “rituximab can be safely recommended for treatment of inflammatory myopathies and myasthenia gravis; however, it is not recommended for the treatment of CIDPN.”
A Japanese study (Tanaka et al) shows the importance of fast of treatment in CIDP at the onset, regardless of the therapeutic approach used. 75.0% of patients treated less than eight weeks after onset and 42.9% of patients treated from eight weeks to one year after onset showed good prognosis, whereas none of the patients treated later than a year after onset demonstrated good prognosis.
Other studies presented at the WCN explore the efficacy of established therapies for the treatment of various subtypes of CIDP. A group from Moldova (Marcoci et al) concludes from a population-based study that intravenous immunoglobulins significantly ameliorate the evolution of all phenotypes of CIDP. Corticosteroids are efficient in the short-term management of CIDP, but not in the treatment of motor phenotype of CIDP. The treatment of corticosteroids in combination with azathioprine is cost-efficient in the treatment of symmetric motor-sensitive and sensitive neuropathies.
Sources: WCN Abstract Hughes et al, Fingolimod (FTY720) oral for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): study design of the phase 3 FORCIDP trial; WCN Abstract Marcoci et al, Immunosuppressive treatment in relation to different phenotypes of clinical presentation of chronic inflammatory demyelinating polyneuropathy (CIDP); WCN Abstract Tanaka et al, Time from onset to treatment and prognosis in patients with CIDP: a 3-year follow-up of 29 cases; WCN Abstract Garneau: Use of Rituxan in neurological disease and outcomes in key disease groups.
WCN Press Office
B&K - Bettschart&Kofler Kommunikationsberatung
Dr Birgit Kofler
Phone: +43-1-319 43 78-13
Mobile: +43-676-63 68 930