Fibrillarin is a highly conserved 34-kD nucleolar protein localized in the fibrillar regions of nucleoli. It is associated with U3 snRNP complex, which is thought to play a role in the processing of pre-ribosomal RNA. Anti-fibrillarin Antibodies may characterize a subset of patients with diffuse sclerosis and widespread telangiectasia. These antibodies are also found in other connective tissue diseases such as mixed connective tissue disease (MCTD), SLE, CREST syndrome, rheumatoid arthritis, and Sjogren’s syndrome. See Extractable Nuclear Antigens (ENA) Antibodies, Serum
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