| Popis: |
Polymyositis (PM) and dermatomyositis (DM) are systemic autoimmune diseases of unknown aetiology in which the skeletal muscles are the main targets (Bohan & Peter, 1975). Despite the improvement obtained in recent years with new therapeutic options, their prognosis remains poor, with higher rates of morbidity and mortality (Dalakas, 1991, 2001). Due to the rarity of the disease, few well-designed studies have been published and, to the best of our knowledge, only five randomised controlled trials have been carried out (Choy, 2002). A low incidence of the disease, a characteristic relapsing/remitting or chronic and persistently active course, a lack of agreed standardised criteria for diagnosis and for assessment of disease activity makes it difficult to carry out and to compare studies. Conventional first line therapy is based on glucocorticoids and their use in many patients requires long-term use to control disease. Many patients suffer from the side effects of glucocorticoids while others can be refractory to first-line therapy. Thus, there is often the need to add immunosuppressive or immunomodulatory agents both to improve the disease’s response and to reduce the risk of long-term complications linked to glucocorticoids (Choy, 2009). Among the treatment options, the use of intravenous immunoglobulin is still matter of debate. In this chapter we describe the use of intravenous immunoglobulin in inflammatory myopathies, revising the literature and reporting our experience. Most of the patients with polymyositis or dermatomyositis receive an immunosuppressant such as azathioprine, methotrexate, cyclosporine A or mycophenolate mofetil. We decided to verify if the use of intravenous immunoglobulin as add-on treatment with cyclosporine A or mycophenolate mofetil could improve the outcome or reduce the rate of side effects that are usually linked to the immunosuppressant. The subcutaneous administration of immunoglobulin could be considered as an alternative to intravenous immunoglobulin. In primary immunodeficiency, subcutaneous immunoglobulin has been demonstrated to be linked to a lower incidence of adverse reactions, with reliable efficacy and improvement in the quality of life of treated subjects. We have been the first to publish a series of seven patients with immune-mediated myopathies treated with subcutaneous immunoglobulin. Here we present data relating to a larger series. Finally, our intention is to review the data related to the mechanisms of action |
