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

Abstract

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.Keywords: immunoglobulin, neuromuscular diseases, autoimmunity, inflammatory myopathy. RESUMONos últimos anos, um número progressivo de doenças neuromusculares passaram a ser reconhecidas tanto por ser causadas por mecanismos autoimunes ou por envolver importantes componentes autoimunes. Os mecanismos fisiopatológicos e as manifestações clínicas envolvidos têm sido mais bem reconhecidos e muitas de tais doenças são potencialmente tratáveis por imunossupressão ou imunomodulação com imunoglobulina intravenosa (IVIg). IVIg vem sendo utilizada em uma variedade de doenças neurológicas imunomediadas, sendo alvo de amplo uso em doenças dos sistemas nervosos central e periférico. Objetivo: Oferecer uma visão global sobre os principais tópicos relacionados aos mecanismos de ação e aos diferentes usos terapêuticos da IVIg na prática neurológica, principalmente em doenças neuromusculares.Palavras-chave: imunoglobulina, doenças neuromusculares, autoimunidade, miopatia inflamatória.

Hydroxychloroquine-Induced Myopathy Responding to Intravenous Immunoglobulin (IVIG)

“…The mechanism of action that is responsible for this improvement is not clear. An anti-inflammatory and/or immunomodulatory effect of the drug might play a role [13]. Our finding suggests that IVIG may attenuate antimalarial-induced myopathy, but it should be used cautiously especially in patients who are at risk for thromboembolic events.…”

Rationale Diagnostik und Therapie von Myopathien

“…Die inflammatorische Komponente der s-IBM scheint daher nicht der beste therapeutische Angriffspunkt zu sein, der zu durchgreifender klinischer Besserung verhilft (29). I.v.-Immunglobuline wurden in Open-label-, doppelblinden und placebokontrollierten Studien mit unterschiedliche Erfahrungsberichten eingesetzt (32,33), sodass aufgrund fehlender klarer Therapieempfehlungen ein sechsmonatiger empirischer Therapieversuch mit Glucocorticoiden, i.v.-Immunglobulinen und Immunsuppression (MTX, AZA) -teils auch in Kombination-diskutiert werden sollte (34).…”