Clinical applications of immunoglobulin in
                    neuromuscular diseases: focus on inflammatory myopathies

Souza, Paulo Victor Sgobbi de; Pinto, Wladimir Bocca Vieira de Rezende; Oliveira, Acary Souza Bulle

doi:10.1590/0004-282X20140160

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Sumário

View and Review • Arq. Neuro-Psiquiatr. 72 (12) • Dec 2014 • https://doi.org/10.1590/0004-282X20140160 copy

Clinical applications of immunoglobulin in neuromuscular diseases: focus on inflammatory myopathies

Aplicações clínicas de imunoglobulina em doenças neuromusculares com foco em miopatias inflamatórias

Authorship SCIMAGO INSTITUTIONS RANKINGS

During recent years, an increasing number of neuromuscular diseases have been recognized either to be caused primarily by autoimmune mechanisms, or to have important autoimmune components. The involved pathophysiological mechanisms and clinical manifestations have been better recognized and many of these disorders are potentially treatable by immunosuppression or by immunomodulation with intravenous immunoglobulin (IVIg). IVIg has been tried in a variety of immune-mediated neurological diseases, being target of widespread use in central and peripheral nervous systems diseases.

Objective

To give an overview of the main topics regarding the mechanism of action and different therapeutic uses of IVIg in neurological practice, mainly in neuromuscular diseases.

immunoglobulin; neuromuscular diseases; autoimmunity; inflammatory myopathy

Clinical diseases and intravenous immunoglobulin use
Hematologic diseases	Idiopathic thrombocytopenic purpura; autoimmune hemolytic anemia; hemolytic disease of the newborn with severe hyperbilirubinaemia; parvovirus B19-associated aplasia; immune neutropenia; thrombotic thrombocytopenic purpura; acquired von Willebrand disease; acquired factor VIII inhibitor; prophylaxis (for graft-versus-host disease and infectious) after bone marrow transplantation (in recipients older than 20 years of age) and for B-cell chronic lymphocytic leukaemia
Rheumatologic diseases	Kawasaki disease, systemic lupus erythematosus (hematological, central and peripheral nervous system, serositis and renal activities), fibromyalgia
Dermatologic diseases	Autoimmune bullous disease (bullous pemphigoid, pemphigus vulgaris and foliaceus, linear IgA disease), erythema multiforme, toxic epidermal necrolysis, chronic urticaria, pyoderma gangrenosum, scleromyxedema, atopic dermatitis
Gastroenterological diseases	Severe Clostridium difficile colitis, inflammatory bowel disease
Primary immunodeficiencies	Prophylaxis in hypogammaglobulinaemia; severe combined Immunodeficiency; X-linked agammaglobulinaemia, common variable immunodeficiency disorders, immunodeficiency with hyper-IgM syndrome, Wiskott-Aldrich syndrome, ataxia-telangiectasia (Louis-Bar syndrome), DiGeorge syndrome, Good syndrome (thymoma with immunodeficiency), X-linked lymphoproliferative syndrome, hyper-IgE syndrome
Miscellaneous	Prophylaxis in children with chronic HIV infection; autoimmune uveitis; Birdshot retinochoroidopathy, streptococcal toxic schock syndrome, systemic vasculitis, severe drug-induced antibody deficiency

Properties	Gammagard	Gammar-P	Gamunex	Octagam	Flebogamma	Sandoglobulina Privigen	Kiovig
Laboratory	ARC/Baxter	Aventis	Bayer	Octapharma	Grifols	CSL Behring	Baxter
Form	Powder	Powder	Liquid	Liquid	Liquid	Liquid	Liquid
Concentration (%)	5-10	5	10	5	5-10	10	10
Osmolarity	125-636	309	258	310-380	327-342	320	309
IgA (µg/mL)	<2.4	<25	46	<0.4	<0.003	<0.025	<0.14
Stabilizer	Glucose (20-40 mg)	Sucrose (50 mg)	None	Maltose	Sorbitol 5%	Proline	Sucrose
Salt (mg/mL)	8.5-17	5	Trace	0	<3.2	<1	5
Time of infusion (hours)	1.2-5	5-6	2	4-6	3.1	1.2-5	5-6
pH	6.4-7.2	6.4-7.2	4-4.5	5.1-6	5.6	4.8	6.4-7.2

Table 3 Major groups of neurological diseases for which intravenous immunoglobulin has been studied in clinical trials, case reports and currently used in practice²2 Elovaara I, Apostolski S, Doorn P, Gilhus NE, Hietaharju A, Honkaniemi J et al. 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. Eur J Neurol. 2008;15(9):893-908. http://dx.doi.org/10.1111/j.1468-1331.2008.02246.x
https://doi.org/10.1111/j.1468-1331.2008... ^,¹⁸18 Hughes RAC, Dalakas MC, Cornblath DR, Latov N, Weksler ME, Relkin N. Clinical applications of intravenous immunoglobulins in neurology. Clin Exp Immunol. 2009;158(Suppl):34-42. http://dx.doi.org/10.1111/j.1365-2249.2009.04025.x
https://doi.org/10.1111/j.1365-2249.2009... ^,²¹21 Dalakas MC. Intravenous immunoglobulin in autoimmune neuromuscular diseases. JAMA. 2004;291(19):2367-75. http://dx.doi.org/10.1001/jama.291.19.2367
https://doi.org/10.1001/jama.291.19.2367... ^,²²22 Stangel M. New advances in the treatment of neurological diseases using high dose intravenous immunoglobulins. Ther Adv Neurol Disord. 2008;1(2):52-61. http://dx.doi.org/10.1177/1756285608095747
https://doi.org/10.1177/1756285608095747... ^,²³23 Gold R, Stangel M, Dalakas MC. Drug Insight: the use of intravenous immunoglobulin in neurology--therapeutic considerations and practical issues. Nat Clin Pract Neurol. 2007;3(1):36-44. http://dx.doi.org/10.1038/ncpneuro0376
https://doi.org/10.1038/ncpneuro0376... ^,²⁴24 Wiles C, Brown P, Chapel H, Guerrini R, Hughes RA, Martin TD et al. Intravenous immunoglobulin in neurological disease: a specialist review. J Neurol Neurosurg Psychiatry. 2002;72(4):440-8. http://dx.doi.org/10.1136/jnnp.72.4.440
https://doi.org/10.1136/jnnp.72.4.440... ^,²⁵25 Cursiefen S, Mäurer M. Current use of immunoglobulins in neurology. Nervenarzt. 2008;79(2 Suppl):67-74. 10.1007/s00115-008-2461-y
https://doi.org/10.1007/s00115-008-2461-... ^,²⁶26 Winkelmann A, Zettl UK. Use of intravenous immunoglobulin in the treatment of immune-mediated demyelinating diseases of the nervous system. Curr Pharm Des. 2012;18(29):4570-82. http://dx.doi.org/10.2174/138161212802502314
https://doi.org/10.2174/1381612128025023... .

Intravenous immunoglobulin and groups of neurological diseases
Neuromuscular Diseases	Inflammatory myopathies (dermatomyositis, polymyositis, inclusion body myositis – sporadic and autosomal recessive hereditary type), neuromuscular junction disorders (myasthenia gravis class II/III Osserman-Genkins classification, myasthenic crisis or exacerbation), peripheral neuropathy and polyradiculoneuropathy (proximal subacute diabetic neuropathy, multifocal motor neuropathy, Guillain-Barré syndrome and variants, Chronic inflammatory demyelinating polyneuropathy, critical illness polyneuropathy and/or myopathy, autoimmune autonomic ganglionopathy), motor neuron disease (post-Polio syndrome), others (complex regional pain syndrome type 1, formerly known as reflex sympathetic dystrophy or Sudeck’s atrophy; resistant neuropathic pain)
Dementia and cognitive disorders	Alzheimer’s disease, Mild cognitive impairment
Cerebellar ataxias	Spinocerebellar ataxias, sporadic cerebellar ataxia (including the cerebellar predominant variant of multiple system atrophy)
Demyelinating diseases	Relapsing-remitting multiple sclerosis, acute disseminated encephalomyelitis, anti-aquaporin 4 antibody positive neuromyelitis optica spectrum disorder, optic neuritis, chronic relapsing inflammatory optic neuropathy (CRION)
Paraneoplastic neurological disorders	Limbic encephalitis (anti-VGKC, anti-Hu, anti-Ma2, anti-CV2/CRMP5), subacute sensory neuronopathy (anti-Hu, anti-CV2/CRMP5), opsoclonus-myoclonus (anti-Ri, anti-Hu, anti-Ma2, anti-amphiphysin), cerebellar paraneoplastic degeneration (anti-Yo, anti-Hu, anti-Tr, anti-CV2/CRMP5, anti-VGCC), Lambert-Eaton myastenic syndrome (anti-VGCC), peripheral nerve hyperexcitability in Isaacs’ syndrome (anti-VGKC)
Miscellaneous	HIV-associated vacuolar myelopathy, Japanese encephalitis, Rasmussen encephalitis, refractory autoimmune epilepsy with partial seizures, Stiff-person syndrome, PANDAS syndrome (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections), ischaemic stroke, Susac’s syndrome, narcolepsy

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[1] Correspondence: Wladimir Bocca Vieira de Rezende Pinto; Departamento de Neurologia e Neurocirurgia, Universidade Federal de São Paulo; Rua Pedro de Toledo, 650; 04023-900 São Paulo SP, Brasil; E-mail: wladimirbvrpinto@gmail.com