DOI Seite / Zitierlink:
https://doi.org/10.11588/diglit.48120#0019
Clinical and Etiopathogenetic Significances of Autoantibodies to the
Cellular Antigens in Systemic Connective Tissue Diseases
M. Akizuki
Department of Medicine, Keio University School of Medicine, Tokyo, Japan
Production of autoantibodies reactive with various cellular constituents is the
immunological hallmark of systemic connective tissue diseases (CTD). The
clinical significances and mechanisms involved in production of these autoan-
tibodies were studied.
The autoantibodies were classified into two major groups: (1) those found in
particular disease entity (anti-dsDNA, Scl-70, centromere/kinetocore, Jo-1) were
designated as marker antibody and (2) those associated with certain clinical ex-
pressions observed in various CTD (anti-SSA/Ro, SSB/La, U1RNP) were defined
as symptomspecific. The multisystem diseases which characterize CTD are ex-
plained by the combination of autoantibodies detected in an individual patient
serum. Demonstration of specific autoantibodies has diagnostic and prognostic
implications. Furthermore, the homogenous groups of patients were identified by
specific antibodies: (1) antiphospholipid antibody syndrome: arterial and venous
thrombotic episodes, recurrent spontaneous abortions (2) anti-aminoacyl-tRNA
synthetase antibody syndrome: PM/DM with chronic interstitial lung disease and
decreased lung volume (shrinking lung). (3) anti-SSB/La: patients with character-
istic cutaneous symptoms.
Simultaneous occurrences of certain sets of autoantibodies gave us an insight
to study the immunological mechanisms involved in autoantibody production. In
addition to the previously known combinations (anti-Sm with anti-UIRNP, anti-
SSB/La with antigroup (HMG) were produced in association with anti-histone
antibodies. It appears that co-existing autoantibodies of CTD are directed to the
exposed components of the same antigenic macromolecules.
Antibodies to the Ul-RNP and SSB/La antigens appear to be produced by re-
stricted B-cell clones and occurrences of cross reactive idiotypes of these autoanti-
bodies were rare. These features are similar to those found in the specific antibodies
elicited in experimental animals by prolonged immunization. Further studies con-
cerning on the antigenic expressions in patients with^CTD are indicated to explore
the mechanisms of autoantibody production and pathogenesis of CTD.
Cellular Antigens in Systemic Connective Tissue Diseases
M. Akizuki
Department of Medicine, Keio University School of Medicine, Tokyo, Japan
Production of autoantibodies reactive with various cellular constituents is the
immunological hallmark of systemic connective tissue diseases (CTD). The
clinical significances and mechanisms involved in production of these autoan-
tibodies were studied.
The autoantibodies were classified into two major groups: (1) those found in
particular disease entity (anti-dsDNA, Scl-70, centromere/kinetocore, Jo-1) were
designated as marker antibody and (2) those associated with certain clinical ex-
pressions observed in various CTD (anti-SSA/Ro, SSB/La, U1RNP) were defined
as symptomspecific. The multisystem diseases which characterize CTD are ex-
plained by the combination of autoantibodies detected in an individual patient
serum. Demonstration of specific autoantibodies has diagnostic and prognostic
implications. Furthermore, the homogenous groups of patients were identified by
specific antibodies: (1) antiphospholipid antibody syndrome: arterial and venous
thrombotic episodes, recurrent spontaneous abortions (2) anti-aminoacyl-tRNA
synthetase antibody syndrome: PM/DM with chronic interstitial lung disease and
decreased lung volume (shrinking lung). (3) anti-SSB/La: patients with character-
istic cutaneous symptoms.
Simultaneous occurrences of certain sets of autoantibodies gave us an insight
to study the immunological mechanisms involved in autoantibody production. In
addition to the previously known combinations (anti-Sm with anti-UIRNP, anti-
SSB/La with antigroup (HMG) were produced in association with anti-histone
antibodies. It appears that co-existing autoantibodies of CTD are directed to the
exposed components of the same antigenic macromolecules.
Antibodies to the Ul-RNP and SSB/La antigens appear to be produced by re-
stricted B-cell clones and occurrences of cross reactive idiotypes of these autoanti-
bodies were rare. These features are similar to those found in the specific antibodies
elicited in experimental animals by prolonged immunization. Further studies con-
cerning on the antigenic expressions in patients with^CTD are indicated to explore
the mechanisms of autoantibody production and pathogenesis of CTD.