title: Clinical diagnosis compared to classification criteria in in a cohort of 54 patients with systemic sclerosis and associated disorders.
abstract: OBJECTIVE
  To compare clinical diagnosis with two validated classification criteria for systemic sclerosis (SSc) in a cohort of Swiss patients with SSc and associated disorders.
  
  
  METHODS
  Charts of 54 patients with SSc and associated disorders were reviewed and compared with data obtained at a thorough clinical examination using a standardised protocol (Raynaud's phenomen [RP], skin involvement, nailfold capillary microscopy and determination of autoantibody pattern).
  
  
  RESULTS
  According to patient records 6 patients had diffuse cutaneous SSc (dcSSc), 23 limited cutaneous SSc (lcSSc) and 20 were not classified. Two patients had mixed connective tissue disease (MCTD) and 3 overlap syndromes. At the time of clinical examination, 7 patients showed dcSSc (6 plus 1 patient originally classified as lcSSc), 26 lcSSc (20 plus 6 originally not classified) and 16 patients had severe RP which was arbitrarily classified as Raynaud's syndrome (RS). 15 of the latter 16 were antinuclear antibody positive and 7 exhibited pathological nailfold capillaries. On the basis of LeRoy and Medsger's criteria, 6 of these patients could be further classified as limited SSc (lSSc). Of 49 sera tested, 14 contained centromere antibodies at clinical examination, 16 Scl-70, 5 RNA-pol, 1 Ku, 12 antibodies with unknown specificity, and one serum was autoantibody negative.
  
  
  CONCLUSIONS
  A substantial number of patients with minor cutaneous manifestations do not fulfil ACR classification criteria, though they have typical clinical signs of SSc. Characteristic features in these patients are presence of Raynaud's phenomenon, antinuclear antibodies and pathological changes in nailfold capillary microscopy. Application of the diagnostic criteria recently proposed by LeRoy and Medsger makes it possible to name many of these patients. The use of these criteria is recommended for clinical management.
authors.affiliation: Department of Rheumatology, Clinical Immunology and Allergology, University Hospital, Bern, Switzerland. hansruedi.ziswiler@insel.ch
authors.forename: Hans-Rudolf
authors.initials: HR
authors.lastname: Ziswiler
authors.forename: Romana
authors.initials: R
authors.lastname: Urech
authors.forename: Judith
authors.initials: J
authors.lastname: Balmer
authors.forename: Monika
authors.initials: M
authors.lastname: Ostensen
authors.forename: Rudolf
authors.initials: R
authors.lastname: Mierau
authors.forename: Peter M
authors.initials: PM
authors.lastname: Villiger
mesh_terms: D000974:Antibodies, Antinuclear; D005260:Female; D006305:Health Status Indicators; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D011928:Raynaud Disease / Q000175:diagnosis / Q000503:physiopathology; D012595:Scleroderma, Systemic / Q000145:classification / Q000175:diagnosis* / Q000503:physiopathology; D012720:Severity of Illness Index; D013557:Switzerland
chemical_list: D000974:Antibodies, Antinuclear