title: Distinctive cutaneous and systemic features associated with antitranscriptional intermediary factor-1γ antibodies in adults with dermatomyositis.
abstract: BACKGROUND
  Antibodies against transcriptional intermediary factor (TIF)-1γ are associated with malignancy in dermatomyositis (DM). Identification of clinical findings associated with anti-TIF-1γ antibodies in DM is a high priority for both patient diagnosis and risk assessment.
  
  
  OBJECTIVE
  We sought to define the clinical phenotype of patients with anti-TIF-1γ DM.
  
  
  METHODS
  Using a novel, sensitive, and specific assay for anti-TIF-1γ antibodies, we retrospectively tested plasma from 134 adult patients with DM and examined associations between anti-TIF-1γ antibodies and particular clinical and laboratory features.
  
  
  RESULTS
  In all, 55 (41%) patients had autoantibodies to TIF-1γ. Anti-TIF-1γ positive patients were less likely to have systemic features including interstitial lung disease, Raynaud phenomenon, and arthritis/arthralgia. Patients with TIF-1γ autoantibodies had more extensive skin involvement, and some patients manifested characteristic findings including palmar hyperkeratotic papules, psoriasis-like lesions and a novel finding of hypopigmented and telangiectatic ("red on white") patches.
  
  
  CONCLUSIONS
  This was a retrospective study from a single tertiary referral center.
  
  
  CONCLUSIONS
  TIF-1γ is the most commonly targeted DM-specific autoantigen in adults in a large US cohort. Although these patients tend to have less systemic involvement, their skin disease is often extensive and characteristic. Recognition of cutaneous findings in anti-TIF-1γ positive patients may allow more accurate and timely diagnosis and effective treatment of patients with DM.
authors.affiliation: Department of Dermatology, Stanford University School of Medicine, Redwood City, California. Electronic address: fiorentino@stanford.edu.
authors.forename: David F
authors.initials: DF
authors.lastname: Fiorentino
authors.affiliation: Department of Dermatology, Stanford University School of Medicine, Redwood City, California.
authors.forename: Karen
authors.initials: K
authors.lastname: Kuo
authors.affiliation: Division of Immunology and Rheumatology, Stanford University School of Medicine, Redwood City, California; Department of Veterans Affairs Palo Alto Health Care System, Palo Alto, California.
authors.forename: Lorinda
authors.initials: L
authors.lastname: Chung
authors.affiliation: Department of Dermatology, Stanford University School of Medicine, Redwood City, California.
authors.forename: Lisa
authors.initials: L
authors.lastname: Zaba
authors.affiliation: Department of Dermatology, Stanford University School of Medicine, Redwood City, California.
authors.forename: Shufeng
authors.initials: S
authors.lastname: Li
authors.affiliation: Johns Hopkins University School of Medicine, Division of Rheumatology, Baltimore, Maryland.
authors.forename: Livia
authors.initials: L
authors.lastname: Casciola-Rosen
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood*; D002648:Child; D002675:Child, Preschool; D003882:Dermatomyositis / Q000097:blood* / Q000175:diagnosis / Q000276:immunology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D014157:Transcription Factors / Q000276:immunology*; D055815:Young Adult
chemical_list: D001323:Autoantibodies; C511956:TRIM33 protein, human; D014157:Transcription Factors
keywords: Cutaneous Dermatomyositis Assessment and Severity Index; autoantibodies; dermatomyositis; malignancy; phenotype; transcriptional intermediary factor-1γ