title: Systemic sclerosis without antinuclear antibodies or Raynaud's phenomenon: a multicentre study in the prospective EULAR Scleroderma Trials and Research (EUSTAR) database.
abstract: OBJECTIVE
  To assess patients with SSc who present without circulating ANAs or RP.
  
  
  METHODS
  Five thousand three hundred and ninety patients who fulfilled the ACR criteria for SSc and were enrolled in the EULAR Scleroderma Trials and Research (EUSTAR) database were screened for the absence of both RP and circulating ANA. To differentiate SSc from its mimics, additional information was gathered using a standardized questionnaire.
  
  
  RESULTS
  Five thousand three hundred and seventy-eight (99.8%) of the 5390 SSc patients in the EUSTAR database had either detectable ANA or a history of RP. Twelve (0.2%) patients lacked both circulating ANA and RP. Details of the medical history could be obtained for seven patients. Three cases were compatible with ANA-negative and RP-negative SSc and were not typical of any known SSc mimic. Four patients had a malignancy: two had breast cancer, one had multiple myeloma with possible scleromyxoedema and one had bladder carcinoma. There was no temporal relationship between the onset of skin fibrosis and that of the tumour. Although no patient with confirmed nephrogenic systemic fibrosis was identified among the cases of ANA-negative and RP-negative SSc, the presentation of one patient could be compatible with that of nephrogenic systemic fibrosis other than for the absence of chronic kidney disease or of known prior gadolinium exposure.
  
  
  CONCLUSIONS
  We have identified a very small subgroup of SSc patients who lack both circulating ANA and RP, none of whom fulfils the diagnostic criteria for any known SSc mimic. Prospective studies are needed to elucidate the clinical presentation, evolution and outcome of such patients.
authors.affiliation: Department of Rheumatology, Basel University, Felix Platter Spital, Basel, Switzerland.
authors.forename: Daniel
authors.initials: D
authors.lastname: Schneeberger
authors.forename: Alan
authors.initials: A
authors.lastname: Tyndall
authors.forename: Jonathan
authors.initials: J
authors.lastname: Kay
authors.forename: Klaus H
authors.initials: KH
authors.lastname: Søndergaard
authors.forename: Patricia E
authors.initials: PE
authors.lastname: Carreira
authors.forename: Ewa
authors.initials: E
authors.lastname: Morgiel
authors.forename: Katrin
authors.initials: K
authors.lastname: Deuschle
authors.forename: Chris T
authors.initials: CT
authors.lastname: Derk
authors.forename: Malgorzata
authors.initials: M
authors.lastname: Widuchowska
authors.forename: Ulrich A
authors.initials: UA
authors.lastname: Walker
mesh_terms: D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000032:analysis*; D016208:Databases, Factual; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D011928:Raynaud Disease / Q000276:immunology*; D045743:Scleroderma, Diffuse / Q000175:diagnosis / Q000276:immunology*
chemical_list: D000974:Antibodies, Antinuclear