title: 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: Department of Pediatrics, Yokohama City University , Kanazawaku, Yokohama , Japan.
authors: Mei
authors: M
authors: Momomura
authors: Takako
authors: T
authors: Miyamae
authors: Tomo
authors: T
authors: Nozawa
authors: Masako
authors: M
authors: Kikuchi
authors: Toshitaka
authors: T
authors: Kizawa
authors: Tomoyuki
authors: T
authors: Imagawa
authors: Laurent
authors: L
authors: Drouot
authors: Fabienne
authors: F
authors: Jouen
authors: Olivier
authors: O
authors: Boyer
authors: Shumpei
authors: S
authors: Yokota
mesh_terms: D000293:Adolescent; D001323:Autoantibodies / Q000097:blood*; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008775:Methylprednisolone / Q000627:therapeutic use*; D009135:Muscular Diseases / Q000097:blood / Q000188:drug therapy / Q000276:immunology* / Q000628:therapy*; D010951:Plasma Exchange; D018271:Signal Recognition Particle / Q000276:immunology*; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents; C480632:SRP54 protein, human; D018271:Signal Recognition Particle; D003520:Cyclophosphamide; D008775:Methylprednisolone