The role of high-dose immune globulin intravenous in the treatment of dermatomyositis

International Immunopharmacology
M C Dalakas

Abstract

Dermatomyositis (DM) is a complement-mediated microangiopathy affecting skin and muscle resulting in skin abnormalities, including subcutenous calcifications, muscle weakness and disability. The disease generally responds to steroids or immunosuppressive drugs, but a number of patients are resistant or partially responsive to these therapies, prompting us to examine the efficacy of IVIg. A double-blind placebo-controlled study demonstrated that IVIg is very effective in improving both the muscle strength and the skin rash. The clinical benefit, which was impressive in patients with early disease, was associated with improvement in the muscle cytoarchitecture. Quantitative histological studies in repeated muscle biopsies showed a statistically significant increase in the size of muscle fibers and the number of capillaries with normalization of the capillary diameter. Resolution of the aberrant immunopathological parameters, including interception of complement activation products and downregulation of T cells, ICAM-I, VCAM, TGF-beta and MHC-I molecules, was also noted. Further, a number of immunoregulatory and structural genes were modified in the patients' muscle biopsies after therapy. The study concluded that IVIg is an effec...Continue Reading

References

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Citations

Jan 2, 2010·Rheumatology International·Mike RecherAdriano Fontana
Jul 25, 2008·International Archives of Medicine·Deepak JoshiPaul Kitchen
Jun 19, 2009·Rheumatology International·Silvia Sánchez-RamónFrancisco Javier López-Longo
Mar 1, 2008·Reumatología clinica·Francisco Javier López Longo
Apr 22, 2008·Journal of the American Academy of Dermatology·Luciano J Iorizzo, Joseph L Jorizzo
Dec 19, 2006·Seminars in Cutaneous Medicine and Surgery·Mary Jo J KernsMichael P Heffernan
Jun 30, 2012·Dermatologic Therapy·Christina Lam, Ruth Ann Vleugels
Aug 28, 2015·International Immunopharmacology·Ning XinYong Zhang
Nov 29, 2001·Journal of Molecular Biology·M G RudolphI A Wilson
May 29, 2018·Saudi Journal of Gastroenterology : Official Journal of the Saudi Gastroenterology Association·Zeyu WangDongqiang Zhao
Apr 3, 2007·Obstetrics and Gynecology
Feb 3, 2007·Obstetrics and Gynecology·Lloyd WilliamsLucy Bayer-Zwirello
Dec 19, 2006·American Journal of Clinical Dermatology·Rhonda D Quain, Victoria P Werth
Feb 19, 2009·Dermatologic Therapy·Leslie Castelo-Soccio, Abby S Van Voorhees
Jul 25, 2007·American Journal of Health-system Pharmacy : AJHP : Official Journal of the American Society of Health-System Pharmacists·David A White, Mandy C Leonard

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