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EFNS Guidelines for the Use of Intravenous Immunoglobulin In European Journal of Neurology 2008, 15: 893–908 doi:10.1111/j.1468-1331.2008.02246.x EFNS TASK FORCE/CME ARTICLE EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases EFNS task force on the use of intravenous immunoglobulin in treatment of neurological diseases Members of the Task Force: I. Elovaaraa, S. Apostolskib, P. van Doornc, N. E. Gilhusd, A. Hietaharjue, J. Honkaniemif, I. N. van Schaikg, N. Scoldingh, P. Soelberg Sørenseni and B. Uddj aDepartment of Neurology and Rehabilitation, Tampere University Hospital and Medical School, University of Tampere, Tampere, Finland; bInstitute of Neurology, School of Medicine, University of Belgrade, Belgrade, Serbia; cDepartment of Neurology, Erasmus Medical Centre, Rotterdam, The Netherlands; dDepartment of Neurology, Haukeland University Hospital, Bergen, Norway; eDepartment of Neurology and Rehabilitation, Tampere University Hospital, Tampere, Finland; fDepartment of Neurology, Vaasa Central Hospital, Vaasa, Finland; gDepartment of Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands; hUniversity of Bristol Institute Of Clinical Neuroscience, Frenchary Hospital UK Bristol, UK; iDepartment of Neurology, National University Hospital, Rigshospitalet, Copenhagen, Denmark; and jDepartment of Neurology and Rehabilitation, Tampere University Hospital and Medical School, University of Tampere, Tampere, Finland Keywords: Despite high-dose intravenous immunoglobulin (IVIG) is widely used in treatment of acute disseminated a number of immune-mediated neurological diseases, the consensus on its optimal use encephalomyelitis, BaloÕs is insufficient. To define the evidence-based optimal use of IVIG in neurology, the concentric sclerosis, child- recent papers of high relevance were reviewed and consensus recommendations are hood refractory epilepsy, given according to EFNS guidance regulations. The efficacy of IVIG has been proven chronic inflammatory in Guillain-Barre´syndrome (level A), chronic inflammatory demyelinating polyradi- demyelinating poly- culoneuropathy (level A), multifocal mononeuropathy (level A), acute exacerbations radiculoneuropathy, der- of myasthenia gravis (MG) and short-term treatment of severe MG (level A recom- matomyositis, Guillain- mendation), and some paraneoplastic neuropathies (level B). IVIG is recommended as Barre´syndrome, intrave- a second-line treatment in combination with prednisone in dermatomyositis (level B) nous immunoglobulin, and treatment option in polymyositis (level C). IVIG should be considered as a second multifocal motor neurop- or third-line therapy in relapsing–remitting multiple sclerosis, if conventional athy, multiple sclerosis, immunomodulatory therapies are not tolerated (level B), and in relapses during myastenia gravis, neuro- pregnancy or post-partum period (good clinical practice point). IVIG seems to have a myelitis optica, Rasmus- favourable effect also in paraneoplastic neurological diseases (level A), stiff-person senÕs encephalitis, stiff- syndrome (level A), some acute-demyelinating diseases and childhood refractory person syndrome and epilepsy (good practice point). post-polio syndrome Received 16 March 2008 Accepted 25 June 2008 diseases of the central and peripheral nervous system. Background Although underlying mechanisms of action of IVIG Intravenous immunoglobulin (IVIG) has been success- have not been fully explained, it is known that IVIG can fully used to treat a number of immune-mediated interfere with the immune system at several levels. The effect of IVIG in one of particular diseases may not be Correspondence: Prof. Irina Elovaara, Department of Neurology and attributed to only one of its mechanisms of action, Rehabilitation, Tampere University Hospital and Medical School, University of Tampere, Tampere, Finland (tel.: 358 3 311 66692; because the pathophysiology of these diseases is com- fax: 358 3 311 64351; e-mail: irina.elovaara@uta.fi). plex. IVIG has been used as a first-line therapy in Guillain-Barre´syndrome (GBS), chronic inflammatory This is a Continuing Medical Education article, and can be found with corresponding questions on the internet at http://www.efns.org/ demyelinating polyradiculoneuropathy (CIDP), content.php?pid=132. Certificates for correctly answering the multifocal motor neuropathy (MMN) and dermato- questions will be issued by the EFNS. myositis (DM). It may be used also in diseases of Ó 2008 The Author(s) Journal compilation Ó 2008 EFNS 893 894 I. Elovaara et al. neurotransmission, multiple sclerosis (MS) and in some production; they also modulate anti-idiotypic networks rare neurological disorders of adults and children vital to immune tolerance. including RasmussenÕs encephalitis (RE), stiff-person In addition, IVIG contains anti-idiotypic antibodies syndrome (SPS) and post-polio syndrome (PPS). In this that bind to F(ab) to neutralize autoantibodies – a paper we have reviewed the available literature on the mechanism involved in GM1-related neuropathy and use of IVIG in treatment of neurological diseases and perhaps GBS [12,13]. Finally considering cytopathic are offering evidence-based recommendations for its use immune effectors, IVIG interferes with the complement in these disorders. system: the beneficial effects of IVIG are associated with disappearance of complement in the muscles [14], involved suppression of macrophage function through Materials and methods induction of increased U FccRII-B expression, reducing phagocytic activity. Search strategy The task force systematically searched Ovid Medline and Guillain-Barre´ syndrome several other sources to a set of predefined key question. The final search was performed in December 2007. Re- The proposed autoimmune aetiology led to the intro- cent papers of high relevance were reviewed. Consensus duction of immunotherapy. Before its introduction, was reached by discussions during a task force meeting. 10% of patients died and 20% were left seriously dis- Evidence was classified as class I–IV and recommenda- abled [15]. Plasma exchange (PE) was introduced as a tions as level A–C according to the current EFNS possible treatment in 1978 and was shown to offer sig- guidelines [1]. When only class IV evidence was available nificant benefit by a randomized trial published in 1985 the task force has offered advice as good practice points. [16,17]. It became the gold standard against which other treatments were measured [18]. Intravenous immunoglobulin was introduced for Mechanisms of action of IVIG in neurological GBS in 1988 [19]. In 1992, the first randomized trial diseases comparing IVIG and PE showed similar effects from Despite over 25 yearsÕ usage in autoimmunity, how each treatment [20]. In five trials with altogether 582 concentrated non-host immunoglobulins, delivered participants, the improvement on the disability grade intravenously, produce their clinical effect remains un- scale with IVIG was very similar to that with PE, known. Of many potential mechanisms of action [2], WMD 20.02 (95% CI 20.25–0.20) [20–24]. This effec- whether one (unlikely), all (likewise) or several together tiveness of IVIG has been shown in GBS patients un- are important remains obscure. Probably, different ef- able to walk unaided (GBS disability score ‡3) who fects are relevant in different disorders. We here con- were started on IVIG within the first 2 weeks after sider the range of possible actions of IVIG, stressing onset of weakness. Results from PE studies indicate effects that appear especially pertinent in specific neu- that PE is also effective when applied in patients less rological disorders. severely affected [25] and in patients who are treated The possibility that IVIG works through non-im- within the first 4 weeks from onset [17]. This has not mune mechanisms [3] – e.g. binding and removing been investigated in studies on IVIG treatment. Al- microbial toxins, or targeting their surface antigens – is though PE was more frequently discontinued, there was perhaps less relevant in neurology. However, direct also no significant difference between IVIG and PE for actions on oligodendrocyte progenitors have been other outcome measures [23]. One trial compared PE postulated to explain an effect in promoting experi- alone with PE followed by IVIG: the 128 patients who mental remyelination [4,5], although alternative mech- received both treatments did not had significant extra anisms are possible [6–9]. benefit after 4 weeks of treatment compared with the More direct immune-modulating effects are generally 121 patients who received PE alone [22]. considered more neurologically relevant. T-cell prolifer- In children, who may have a better prognosis than ation is reduced by IVIG [10], various pro-inflammatory adults, limited evidence from three open trials suggests cytokines are suppressed, including interleukin-1, that IVIG hastens recovery compared with supportive tumour necrosis factor-a and c-interferon, and lym- care alone [26–28], which is supported by a good quality phocyte and monocyte apoptosis is induced [11]. observational study [29]. Endogenous immunoglobulin production and B-cell A recent trial reported possible minor short-term differentiation are suppressed, and IgG catabolism is benefit when high-dose intravenous methylprednisolone accelerated by IVIG [3]. Therapeutic immunoglobulins was combined with IVIG [30]. The significance of this exert Fc region-mediated inhibition of antibody benefit has been debated [31]. Ó 2008 The Author(s) Journal compilation Ó 2008 EFNS European Journal of Neurology 15,
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