Serum levels of anti-SRP54 antibodies reflect disease activity of necrotizing myopathy in a child treated effectively with combinatorial methylprednisolone pulses and plasma exchanges followed by intravenous cyclophosphamide.

Abstract

We describe the effectiveness of combinatorial therapy with plasma exchanges and methylprednisolone pulses followed by intravenous cyclophosphamide in a young girl with anti-signal recognition particle 54 (SRP54) antibody-associated myopathy. We also use a newly described quantitative assay to demonstrate the close association between the titers of anti-SRP54 antibodies and disease activity. This is the first report of a pediatric patient indicating that the serum levels of anti-SRP54 antibodies are also beneficial for monitoring the disease activity of progressive necrotizing myopathy.

• Authors: Mei Momomura, Takako Miyamae, Tomo Nozawa, Masako Kikuchi, Toshitaka Kizawa, Tomoyuki Imagawa, Laurent Drouot, Fabienne Jouen, Olivier Boyer, Shumpei Yokota
• Journal: Modern rheumatology (Mod Rheumatol) Vol. 24 Issue 3 Pg. 529-31 (May 2014) ISSN: 1439-7609 [Electronic] England
• PMID: 24506571 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Autoantibodies
• Immunosuppressive Agents
• SRP54 protein, human
• Signal Recognition Particle
• Cyclophosphamide
• Methylprednisolone

Topics

• Adolescent
• Autoantibodies (blood)
• Combined Modality Therapy
• Cyclophosphamide (therapeutic use)
• Female
• Humans
• Immunosuppressive Agents (therapeutic use)
• Methylprednisolone (therapeutic use)
• Muscular Diseases (blood, drug therapy, immunology, therapy)
• Plasma Exchange
• Signal Recognition Particle (immunology)
• Treatment Outcome