Treatment of Guillain-Barré syndrome and CIDP.
ABSTRACT Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating poly-(radiculo)neuropathy (CIDP) are immune-mediated disorders with a variable duration of progression and a range in severity of weakness. Infections can trigger GBS and exacerbate CIDP. Anti-ganglioside antibodies are important, but there is debate on the role of genetic factors in the pathogenesis of these disorders. Randomized controlled trials (RCT) have shown that intravenous immunoglobulin (IVIg) and plasma exchange (PE) are effective in both GBS and CIDP. Most CIDP patients also improve after steroid therapy. Despite current treatment options, many patients have residual deficits or need to be treated for a long period of time. Therefore, new treatment trials are highly indicated. This review focuses on the current and possible new treatment options that could be guided by recent results from laboratory experiments.
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ABSTRACT: Intravenous immunoglobulin (IVIg) is a proven effective treatment for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and Guillain-Barré syndrome (GBS). In GBS, patients show a large variability in serum immunoglobulin G (IgG) levels after standard IVIg treatment and a large increase in serum IgG levels (ΔIgG) was associated with a better outcome. Whether this is also the case in CIDP is not known. In contrast to GBS, most patients with CIDP need regular IVIg treatment for a prolonged period of time but the speed and magnitude of clinical response varies considerably between patients. Some patients with CIDP may need at least two IVIg courses before clinical signs of improvement become clear. At present, this clinical response is the only indicator used to adjust the IVIg dose and interval during maintenance treatment. Biomarkers reflecting disease activity or IVIg pharmacokinetics might be helpful to monitor patients and find the optimal dosage and frequency of IVIg treatment for individual patients. A recent prospective study in CIDP indicated that the increased ΔIgG after standard IVIg dosage during maintenance treatment was relatively constant within individual patients, but differed considerably between patients who were treated with the same stable dosage and interval of IVIg. Further studies are required to determine whether this variation in pharmacokinetics of IVIg is related with clinical recovery and whether IgG levels can be used as biomarkers to monitor and to adjust the optimal IVIg dosage in individual patients with CIDP.Journal of the Peripheral Nervous System 06/2011; 16 Suppl 1:38-40. · 2.57 Impact Factor
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ABSTRACT: Fas is a transmembrane receptor involved in the death program of several cell lines, including T lymphocytes. Deleterious mutations hitting genes involved in the Fas pathway cause the autoimmune lymphoprolipherative syndrome (ALPS). Moreover, defective Fas function is involved in the development of common autoimmune diseases, including autoimmune syndromes hitting the nervous system, such as multiple sclerosis (MS) and chronic inflammatory demyelinating polyneuropathy (CIDP). In this review, we first explore some peculiar aspects of Fas mediated apoptosis in the central versus peripheral nervous system (CNS, PNS); thereafter, we analyze what is currently known on the role of T cell apoptosis in both MS and CIDP, which, in this regard, may be seen as two faces of the same coin. In fact, we show that, in both diseases, defective Fas mediated apoptosis plays a crucial role favoring disease development and its chronic evolution.Autoimmunity reviews 04/2012; · 6.37 Impact Factor