Autoantibodies to fibrillarin in systemic sclerosis (scleroderma). An immunogenetic, serologic, and clinical analysis

Arthritis Rheum. 1996 Jul;39(7):1151-60. doi: 10.1002/art.1780390712.


Objective: To determine the frequency, clinical associations, and any major histocompatibility complex correlations of antifibrillarin antibodies in patients with systemic sclerosis (SSc).

Methods: Antifibrillarin antibodies were determined by indirect immunofluorescence, immunoblotting, and immunoprecipitation, and HLA class II alleles by DNA oligotyping, in a large cohort of SSc patients.

Results: Antifibrillarin was found in 8% of 335 SSc sera and was significantly more common in blacks (16%) than whites (5%), in males (33%) than females (14%), and in patients with cardiac, renal, or gut involvement. The HLA class II haplotype DRB1*1302, DQB1*0604 was found significantly more frequently in SSc patients with antifibrillarin compared with race-matched normal controls and 260 SSc patients without antifibrillarin. In addition, 1 or more of the HLA-DQB1 alleles *0604, *0301, *0602, and/or *0302 was found in all antifibrillarin-positive patients, and 62% of the antifibrillarin-positive patients had 2 of these HLA-DQB1 alleles, a highly significant difference from both race-matched normal controls and antifibrillarin-negative SSc patients.

Conclusion: Antifibrillarin, although an infrequent nucleolar autoantibody, is a marker for severe SSc, especially in blacks and males, and is strongly associated with a unique HLA haplotype, as well as with combinations of certain HLA-DQB1 alleles.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Alleles
  • Autoantibodies / blood*
  • Base Sequence
  • Black People / genetics
  • Chromosomal Proteins, Non-Histone / immunology*
  • Cohort Studies
  • Female
  • Genes, MHC Class II / genetics*
  • Haplotypes
  • Humans
  • Male
  • Molecular Sequence Data
  • North America / ethnology
  • Scleroderma, Systemic / complications
  • Scleroderma, Systemic / ethnology
  • Scleroderma, Systemic / immunology*
  • Statistics as Topic
  • White People / genetics


  • Autoantibodies
  • Chromosomal Proteins, Non-Histone
  • fibrillarin