Treatment of idiopathic Inflammatory myopathies
Treatment of idiopathic Inflammatory myopathies
Dátum
2014-06-27T13:27:37Z
Szerzők
Bercovici, Einav
Folyóirat címe
Folyóirat ISSN
Kötet címe (évfolyam száma)
Kiadó
Absztrakt
Idiopathic inflammatory myopathies are a group of rare, disorders with the primary features of muscle weakness and inflammatory lesions identified in skeletal muscle specimens. The IIM subtypes are, polymyositis(PM), dermatomyositis (DM) and inclusion body myositis (IBM).
The leading pathomechanism theory for DM, is an autoimmune disorder involving deposition of autoantibodies in the microvasculature, which leads to complement activation and subsequently to capillary necrosis and a mixed leukocytic infiltration . PM pathological findings are characterized by infiltration of CD8+ lymphocytes invading muscle fibers with degenerating and regenerating fibers. IBM is associated with changes identical to PM, but also demonstrates lined vacuoles and deposits of beta-amyloid and ubiquitin.
DM and PM may occur in isolation or in connection with a connective tissue disease or cancer. The fundamental, of therapy is corticosteroids with the addition of other immunosuppressives in severe or refractory disease or patients with intolerable side effects.
The future of IIM treatment includes developing drugs in the field of biology pharmacology. It is a challenging task duo to the fact that relatively little is known about the diseases, and only a few trails where published concerning the long period of follow up on a specific treatment for IIM . Biologics therapies might play a key role in the management of IIM and hopefully achieves better results than conventional therapy.
So far, the most encouraging evidence originates from the anti-CD20 therapy with satisfactory results reported, but this option still requires further investigation.
Leírás
Kulcsszavak
myopathies, Inflammatory myopathies