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Autoantibodies in scleroderma

Junichi Kaburaki, Takeshi Tojo, Mitsuo Honma

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Abstract

Profiles of autoantibodies in patients with scleroderma were reviewed and their possible clinical significance was discussed in this paper.Many autoantibodies including antinuclear antibodies and rheumatoid factors are detected in patients with scleroderma, especially in those with hypergammaglobulinemia. Fluorescent antinuclear antibodies were found in 36-97% of the serum of patients with scleroderma. The specificities of these antinuclear antibodies have been actively investigated in various laboratories using various nuclear antigens and many specificities were recently identified. Among them, two new antibody systems; anti-Og (Scl-1/Scl-70) antibody and anti-centromere antibody, were found to be highly specific for this disorder. Anti-Og antibody was detected in 33% of scleroderma patients and the patients with this antibody tended to have relatively advanced dermal sclerosis and pulmonary fibrosis. Anti-centromere antibody was reported to be present in the serum of patients mainly with CREST syndrome. Other antinulcear antibody systems; e.g. anti-DNA, anti-RNP, and anti-SS·B, were also seen in patients with scleroderma. However, when the patients with overlap syndrome were excluded, anti-RNP antibody was major antibody system in scleroderma. In contrast to the patients with anti-Og antibody, those with antiRNP antibody tended to have much milder sclerodermatous skin change and better prognosis.The presence of the serum autoantibodies in scleroderma patients and the association of these antibodies to patient's clinical characteristics suggest that these antibodies may play some significant roles in developping clinical features and/or might be closely related to the pathogenesis of this disease. Therefore it seems to be important to investigate these antibodies in order to clarify the pathogenesis of scleroderma.

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Profiles of autoantibodies in patients with scleroderma were reviewed and their possible clinical significance was discussed in this paper.Many autoantibodies including antinuclear antibodies and rheumatoid factors are detected in patients with scleroderma, especially in those with hypergammaglobulinemia. Fluorescent antinuclear antibodies were found in 36-97% of the serum of patients with scleroderma. The specificities of these antinuclear antibodies have been actively investigated in various laboratories using various nuclear antigens and many specificities were recently identified. Among them, two new antibody systems; anti-Og (Scl-1/Scl-70) antibody and anti-centromere antibody, were found to be highly specific for this disorder. Anti-Og antibody was detected in 33% of scleroderma patients and the patients with this antibody tended to have relatively advanced dermal sclerosis and pulmonary fibrosis. Anti-centromere antibody was reported to be present in the serum of patients mainly with CREST syndrome. Other antinulcear antibody systems; e.g. anti-DNA, anti-RNP, and anti-SS·B, were also seen in patients with scleroderma. However, when the patients with overlap syndrome were excluded, anti-RNP antibody was major antibody system in scleroderma. In contrast to the patients with anti-Og antibody, those with antiRNP antibody tended to have much milder sclerodermatous skin change and better prognosis.The presence of the serum autoantibodies in scleroderma patients and the association of these antibodies to patient's clinical characteristics suggest that these antibodies may play some significant roles in developping clinical features and/or might be closely related to the pathogenesis of this disease. Therefore it seems to be important to investigate these antibodies in order to clarify the pathogenesis of scleroderma.

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Available abstract

Profiles of autoantibodies in patients with scleroderma were reviewed and their possible clinical significance was discussed in this paper.Many autoantibodies including antinuclear antibodies and rheumatoid factors are detected in patients with scleroderma, especially in those with hypergammaglobulinemia. Fluorescent antinuclear antibodies were found in 36-97% of the serum of patients with scleroderma. The specificities of these antinuclear antibodies have been actively investigated in various laboratories using various nuclear antigens and many specificities were recently identified. Among them, two new antibody systems; anti-Og (Scl-1/Scl-70) antibody and anti-centromere antibody, were found to be highly specific for this disorder. Anti-Og antibody was detected in 33% of scleroderma patients and the patients with this antibody tended to have relatively advanced dermal sclerosis and pulmonary fibrosis. Anti-centromere antibody was reported to be present in the serum of patients mainly with CREST syndrome. Other antinulcear antibody systems; e.g. anti-DNA, anti-RNP, and anti-SS·B, were also seen in patients with scleroderma. However, when the patients with overlap syndrome were excluded, anti-RNP antibody was major antibody system in scleroderma. In contrast to the patients with anti-Og antibody, those with antiRNP antibody tended to have much milder sclerodermatous skin change and better prognosis.The presence of the serum autoantibodies in scleroderma patients and the association of these antibodies to patient's clinical characteristics suggest that these antibodies may play some significant roles in developping clinical features and/or might be closely related to the pathogenesis of this disease. Therefore it seems to be important to investigate these antibodies in order to clarify the pathogenesis of scleroderma.

Key concepts: Anti-nuclear antibody, Autoantibody, Scleroderma (fungus), CREST Syndrome, Antibody, Medicine, Immunology, Extractable nuclear antigens

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