Prevalence and clinical associations of anti-Ku antibodies in patients with systemic sclerosis: A European EUSTAR-initiated multi-centre case-control study

B. Rozman, S. Čučnik, S. Sodin-Semrl, L. Czirják, C. Varjú, O. Distler, D. Huscher, M. Aringer, G. Steiner, M. Matucci-Cerinić, S. Guiducci, B. Stamenković, A. Stanković, T. Kveder

Research output: Contribution to journalArticle

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Abstract

Objectives: To determine the prevalence of anti-Ku antibodies in 625 patients with systemic sclerosis (SSc) from six European rheumatological centres and to evaluate their clinical and serological characteristics. Methods: Sera of 625 consecutive patients with either limited cutaneous or diffuse cutaneous SSc were tested for antibodies to Ku antigen together with other extractable nuclear antigens by counterimmunoelectrophoresis. A case-control design with calculation of bootstrap 95% confidence intervals derived from anti-Ku negative control patients was used to evaluate clinical associations of anti-Ku antibodies. Sera from anti-Ku positive patients with SSc and a control group were additionally tested by immunofluorescence on Hep-2 cell substrates and line immunoassay. Results: Anti-Ku antibodies were found in the sera of 14/625 (2.2%) patients with SSc. Of 14 anti-Ku positive patients with SSc, 10 had no other anti-extractable nuclear antigen (ENA) antibodies detected by counter-immunoelectrophoresis. Using a case-control study design, anti-Ku antibodies were significantly associated with musculoskeletal manifestations such as clinical markers of myositis, arthritis and joint contractures. In addition, a significant negative correlation of anti-Ku antibodies was found with vascular manifestation such as fingertip ulcers and teleangiectasias. There was a striking absence of anti-centromere antibodies as well as anti-polymyositis (PM)/scleroderma (Scl) antibodies in patients that were anti-Ku positive. As expected, anti-Scl70 and punctate nucleolar immunofluorescence patterns were present only in single cases. Conclusion: This is the largest cohort to date focusing on the prevalence of anti-Ku antibodies in patients with SSc. The case-control approach was able to demonstrate a clinically distinct subset of anti-Ku positive patients with SSc with only relative clinical differences in skeletal features. However, the notable exceptions were signs of myositis. This shows the importance of anti-Ku antibody detection for the prediction of this specific clinical subset.

Original languageEnglish
Pages (from-to)1282-1286
Number of pages5
JournalAnnals of the Rheumatic Diseases
Volume67
Issue number9
DOIs
Publication statusPublished - Sep 2008

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Systemic Scleroderma
Case-Control Studies
Anti-Idiotypic Antibodies
Antibodies
Myositis
Fluorescent Antibody Technique
Serum
Counterimmunoelectrophoresis
Diffuse Scleroderma
Polymyositis
Nuclear Antigens
Immunoelectrophoresis
Centromere
Contracture
Set theory
Immunoassay
Ulcer
Arthritis
Blood Vessels
Joints

ASJC Scopus subject areas

  • Rheumatology
  • Immunology

Cite this

Prevalence and clinical associations of anti-Ku antibodies in patients with systemic sclerosis : A European EUSTAR-initiated multi-centre case-control study. / Rozman, B.; Čučnik, S.; Sodin-Semrl, S.; Czirják, L.; Varjú, C.; Distler, O.; Huscher, D.; Aringer, M.; Steiner, G.; Matucci-Cerinić, M.; Guiducci, S.; Stamenković, B.; Stanković, A.; Kveder, T.

In: Annals of the Rheumatic Diseases, Vol. 67, No. 9, 09.2008, p. 1282-1286.

Research output: Contribution to journalArticle

Rozman, B, Čučnik, S, Sodin-Semrl, S, Czirják, L, Varjú, C, Distler, O, Huscher, D, Aringer, M, Steiner, G, Matucci-Cerinić, M, Guiducci, S, Stamenković, B, Stanković, A & Kveder, T 2008, 'Prevalence and clinical associations of anti-Ku antibodies in patients with systemic sclerosis: A European EUSTAR-initiated multi-centre case-control study', Annals of the Rheumatic Diseases, vol. 67, no. 9, pp. 1282-1286. https://doi.org/10.1136/ard.2007.073981
Rozman, B. ; Čučnik, S. ; Sodin-Semrl, S. ; Czirják, L. ; Varjú, C. ; Distler, O. ; Huscher, D. ; Aringer, M. ; Steiner, G. ; Matucci-Cerinić, M. ; Guiducci, S. ; Stamenković, B. ; Stanković, A. ; Kveder, T. / Prevalence and clinical associations of anti-Ku antibodies in patients with systemic sclerosis : A European EUSTAR-initiated multi-centre case-control study. In: Annals of the Rheumatic Diseases. 2008 ; Vol. 67, No. 9. pp. 1282-1286.
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abstract = "Objectives: To determine the prevalence of anti-Ku antibodies in 625 patients with systemic sclerosis (SSc) from six European rheumatological centres and to evaluate their clinical and serological characteristics. Methods: Sera of 625 consecutive patients with either limited cutaneous or diffuse cutaneous SSc were tested for antibodies to Ku antigen together with other extractable nuclear antigens by counterimmunoelectrophoresis. A case-control design with calculation of bootstrap 95{\%} confidence intervals derived from anti-Ku negative control patients was used to evaluate clinical associations of anti-Ku antibodies. Sera from anti-Ku positive patients with SSc and a control group were additionally tested by immunofluorescence on Hep-2 cell substrates and line immunoassay. Results: Anti-Ku antibodies were found in the sera of 14/625 (2.2{\%}) patients with SSc. Of 14 anti-Ku positive patients with SSc, 10 had no other anti-extractable nuclear antigen (ENA) antibodies detected by counter-immunoelectrophoresis. Using a case-control study design, anti-Ku antibodies were significantly associated with musculoskeletal manifestations such as clinical markers of myositis, arthritis and joint contractures. In addition, a significant negative correlation of anti-Ku antibodies was found with vascular manifestation such as fingertip ulcers and teleangiectasias. There was a striking absence of anti-centromere antibodies as well as anti-polymyositis (PM)/scleroderma (Scl) antibodies in patients that were anti-Ku positive. As expected, anti-Scl70 and punctate nucleolar immunofluorescence patterns were present only in single cases. Conclusion: This is the largest cohort to date focusing on the prevalence of anti-Ku antibodies in patients with SSc. The case-control approach was able to demonstrate a clinically distinct subset of anti-Ku positive patients with SSc with only relative clinical differences in skeletal features. However, the notable exceptions were signs of myositis. This shows the importance of anti-Ku antibody detection for the prediction of this specific clinical subset.",
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AU - Sodin-Semrl, S.

AU - Czirják, L.

AU - Varjú, C.

AU - Distler, O.

AU - Huscher, D.

AU - Aringer, M.

AU - Steiner, G.

AU - Matucci-Cerinić, M.

AU - Guiducci, S.

AU - Stamenković, B.

AU - Stanković, A.

AU - Kveder, T.

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N2 - Objectives: To determine the prevalence of anti-Ku antibodies in 625 patients with systemic sclerosis (SSc) from six European rheumatological centres and to evaluate their clinical and serological characteristics. Methods: Sera of 625 consecutive patients with either limited cutaneous or diffuse cutaneous SSc were tested for antibodies to Ku antigen together with other extractable nuclear antigens by counterimmunoelectrophoresis. A case-control design with calculation of bootstrap 95% confidence intervals derived from anti-Ku negative control patients was used to evaluate clinical associations of anti-Ku antibodies. Sera from anti-Ku positive patients with SSc and a control group were additionally tested by immunofluorescence on Hep-2 cell substrates and line immunoassay. Results: Anti-Ku antibodies were found in the sera of 14/625 (2.2%) patients with SSc. Of 14 anti-Ku positive patients with SSc, 10 had no other anti-extractable nuclear antigen (ENA) antibodies detected by counter-immunoelectrophoresis. Using a case-control study design, anti-Ku antibodies were significantly associated with musculoskeletal manifestations such as clinical markers of myositis, arthritis and joint contractures. In addition, a significant negative correlation of anti-Ku antibodies was found with vascular manifestation such as fingertip ulcers and teleangiectasias. There was a striking absence of anti-centromere antibodies as well as anti-polymyositis (PM)/scleroderma (Scl) antibodies in patients that were anti-Ku positive. As expected, anti-Scl70 and punctate nucleolar immunofluorescence patterns were present only in single cases. Conclusion: This is the largest cohort to date focusing on the prevalence of anti-Ku antibodies in patients with SSc. The case-control approach was able to demonstrate a clinically distinct subset of anti-Ku positive patients with SSc with only relative clinical differences in skeletal features. However, the notable exceptions were signs of myositis. This shows the importance of anti-Ku antibody detection for the prediction of this specific clinical subset.

AB - Objectives: To determine the prevalence of anti-Ku antibodies in 625 patients with systemic sclerosis (SSc) from six European rheumatological centres and to evaluate their clinical and serological characteristics. Methods: Sera of 625 consecutive patients with either limited cutaneous or diffuse cutaneous SSc were tested for antibodies to Ku antigen together with other extractable nuclear antigens by counterimmunoelectrophoresis. A case-control design with calculation of bootstrap 95% confidence intervals derived from anti-Ku negative control patients was used to evaluate clinical associations of anti-Ku antibodies. Sera from anti-Ku positive patients with SSc and a control group were additionally tested by immunofluorescence on Hep-2 cell substrates and line immunoassay. Results: Anti-Ku antibodies were found in the sera of 14/625 (2.2%) patients with SSc. Of 14 anti-Ku positive patients with SSc, 10 had no other anti-extractable nuclear antigen (ENA) antibodies detected by counter-immunoelectrophoresis. Using a case-control study design, anti-Ku antibodies were significantly associated with musculoskeletal manifestations such as clinical markers of myositis, arthritis and joint contractures. In addition, a significant negative correlation of anti-Ku antibodies was found with vascular manifestation such as fingertip ulcers and teleangiectasias. There was a striking absence of anti-centromere antibodies as well as anti-polymyositis (PM)/scleroderma (Scl) antibodies in patients that were anti-Ku positive. As expected, anti-Scl70 and punctate nucleolar immunofluorescence patterns were present only in single cases. Conclusion: This is the largest cohort to date focusing on the prevalence of anti-Ku antibodies in patients with SSc. The case-control approach was able to demonstrate a clinically distinct subset of anti-Ku positive patients with SSc with only relative clinical differences in skeletal features. However, the notable exceptions were signs of myositis. This shows the importance of anti-Ku antibody detection for the prediction of this specific clinical subset.

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