title: Disease course and therapeutic approach in dermatomyositis: A four-center retrospective study of 100 patients.
abstract: Dermatomyositis is a life-altering inflammatory disorder of skin and muscle. Details regarding the natural course of this disorder, the effects of specific therapies on its progression, and the optimal therapeutic dosage and duration of prednisone are limited. We performed a retrospective medical record review of dermatomyositis patients at four medical centers. All patients were over the age of 21 and had a clinical diagnosis of dermatomyositis with pathological confirmation. We reviewed average muscle strength, corticosteroid use, creatine kinase levels, and supplemental immunosuppressant use during the 36-month period following each patient's initial assessment. One hundred patients participated with an average age of 50.1 years. Average muscle strength improved and prednisone requirements lessened six months after initial assessment. There was no difference in the mean change in muscle strength or cumulative corticosteroid use over 36 months among those initially treated with methotrexate, mycophenolate mofetil, pulse IVIG, or azathioprine. There was a 5% mortality rate in dermatomyositis patients due to infections. Treated dermatomyositis patients demonstrate the most significant improvement in strength during the first six-to-twelve months following their initial clinical assessment. Additional prospective studies are needed to determine the relative benefit of select immunosuppressant agents in preserving strength and reducing corticosteroid use in dermatomyositis.
authors.affiliation: Department of Neurology, University of Utah, Salt Lake City, UT, USA; Department of Neurology, University of Rochester, Rochester, NY, USA. Electronic address: Nicholas.johnson@hsc.utah.edu.
authors.forename: Nicholas E
authors.initials: NE
authors.lastname: Johnson
authors.affiliation: Department of Neurology, Ohio State University, Columbus, OH, USA.
authors.forename: W David
authors.initials: WD
authors.lastname: Arnold
authors.affiliation: Department of Biostatistics and Computational Biology, University of Rochester, Rochester, NY, USA.
authors.forename: Donald
authors.initials: D
authors.lastname: Hebert
authors.affiliation: Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: Kelly
authors.initials: K
authors.lastname: Gwathmey
authors.affiliation: Department of Neurology, University of Kansas Medical Center, Kansas City, KS, USA.
authors.forename: Mazen M
authors.initials: MM
authors.lastname: Dimachkie
authors.affiliation: Department of Neurology, University of Kansas Medical Center, Kansas City, KS, USA.
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Barohn
authors.affiliation: Department of Neurology, University of Kansas Medical Center, Kansas City, KS, USA.
authors.forename: April L
authors.initials: AL
authors.lastname: McVey
authors.affiliation: Department of Neurology, University of Kansas Medical Center, Kansas City, KS, USA.
authors.forename: Mamatha
authors.initials: M
authors.lastname: Pasnoor
authors.affiliation: Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: Anthony A
authors.initials: AA
authors.lastname: Amato
authors.affiliation: Department of Neurology, University of Rochester, Rochester, NY, USA; Department of Biostatistics and Computational Biology, University of Rochester, Rochester, NY, USA.
authors.forename: Michael P
authors.initials: MP
authors.lastname: McDermott
authors.affiliation: Department of Neurology, Ohio State University, Columbus, OH, USA.
authors.forename: John
authors.initials: J
authors.lastname: Kissel
authors.affiliation: Department of Neurology, University of Rochester, Rochester, NY, USA.
authors.forename: Chad R
authors.initials: CR
authors.lastname: Heatwole
mesh_terms: D000893:Anti-Inflammatory Agents / Q000008:administration & dosage / Q000627:therapeutic use; D003402:Creatine Kinase / Q000032:analysis; D003882:Dermatomyositis / Q000188:drug therapy* / Q000453:epidemiology* / Q000503:physiopathology; D018450:Disease Progression; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D053580:Muscle Strength / Q000187:drug effects; D011241:Prednisone / Q000627:therapeutic use; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D003402:Creatine Kinase; D011241:Prednisone
keywords: Comparative effectiveness; Dermatomyositis; Immunosuppression; Inflammatory myopathies