%0 Journal Article
%T Treatment of Chronic Inflammatory Demyelinating Polyneuropathy: From Molecular Bases to Practical Considerations
%A Paolo Ripellino
%A Thomas Fleetwood
%A Roberto Cantello
%A Cristoforo Comi
%J Autoimmune Diseases
%D 2014
%I Hindawi Publishing Corporation
%R 10.1155/2014/201657
%X Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disease of the peripheral nervous system, in which both cellular and humoral immune responses are involved. The disease is clinically heterogeneous with some patients displaying pure motor form and others also showing a variable degree of sensory dysfunction; disease evolution may also differ from patient to patient, since monophasic, progressive, and relapsing forms are reported. Underlying such clinical variability there is probably a broad spectrum of molecular dysfunctions that are and will be the target of therapeutic strategies. In this review we first explore the biological bases of current treatments and subsequently we focus on the practical management that must also take into account pharmacoeconomic issues. 1. Introduction Chronic inflammatory demyelinating polyneuropathy (CIDP) is a peripheral nervous system disease that is clinically characterized by symmetrical, proximal, and distal weakness with altered sensation and hyporeflexia or areflexia [1]. Clinical course can be either relapsing remitting (RR), chronic progressive (CP), or monophasic [2]. In rare cases, CIDP displays acute onset and fast deterioration in the early phases, followed by chronic progression. This variant of CIDP, defined as ¡°acute onset CIDP,¡± is difficult to distinguish from Guillain-Barr¨¦ syndrome (GBS) in early disease stages [3]. Epidemiological studies on CIDP report an incidence in Northern Italy around 0.6 cases per 100.000 [4]. Nevertheless, it is probable that the real incidence of CIDP is largely underestimated, due to the variety of clinical presentations and the absence of proper diagnostic markers. For this reason, a diagnosis of CIPD must be taken into consideration while examining any polyneuropathy of unknown cause. CIDP is an autoimmune disorder, as demonstrated by a great deal of evidence [5], such as the finding of inflammation at the site of the lesion [6], response to immunomodulatory treatment [7], and possibly the presence of autoantibodies against myelin antigens [8]. Long-term prognosis of CIDP has been correlated to age at onset, response to treatment, and time from onset to the beginning of treatment: young patients with acute onset are more likely to respond to treatment than elderly ones and proximal impairment has been linked to a better prognosis than distal weakness [9, 10]. The main negative prognostic factors of CIDP are progressive course and axonal degeneration [11]. CIDP and multiple sclerosis (MS) display similarities in clinical course and pathogenesis and
%U http://www.hindawi.com/journals/ad/2014/201657/