Open Access Open Badges Research article

Frequency of disease-associated and other nuclear autoantibodies in patients of the German network for systemic scleroderma: correlation with characteristic clinical features

Rudolf Mierau1*, Pia Moinzadeh2, Gabriela Riemekasten3, Inga Melchers4, Michael Meurer5, Frank Reichenberger6, Michael Buslau78, Margitta Worm9, Norbert Blank10, Rüdiger Hein11, Ulf Müller-Ladner12, Annegret Kuhn1314, Cord Sunderkötter14, Aaron Juche15, Christiane Pfeiffer16, Christoph Fiehn17, Michael Sticherling18, Percy Lehmann19, Rudolf Stadler20, Eckhard Schulze-Lohoff21, Cornelia Seitz22, Ivan Foeldvari23, Thomas Krieg2, Ekkehard Genth24 and Nicolas Hunzelmann2

Author Affiliations

1 Laboratory at Rheumaklinik Aachen, Hauptstrasse 21, Aachen, D-52066, Germany

2 Department of Dermatology and Venerology, University of Cologne, Kerpener Strasse 62, Cologne, D-50937, Germany

3 Department of Rheumatology and Clinical Immunology, Charité Universitätsmedizin, Humboldt University, Charitéplatz 1, Berlin, D-10117, Germany

4 Clinical Research Unit for Rheumatology, University Medical Center Freiburg, Breisacher Strasse 66, Freiburg, D-79106, Germany

5 Department of Dermatology, Dresden University Hospital, Fetscherstrasse 74, Dresden, D-01307, Germany

6 Department of Internal Medicine II, University of Giessen, Klinikstrasse 33, Giessen, D-35392, Germany

7 Clinic for Rheumatology, Schneckenhalde 13, Bad Säckingen, D-79713, Germany

8 Reha-Rheinfelden, Salinenstrasse 98, Rheinfelden, CH-4310, Switzerland

9 Department of Dermatology, Venerology and Allergology, Charité Universitätsmedizin, Humboldt University, Charitéplatz 1, Berlin, D-10117, Germany

10 Department of Internal Medicine V, University of Heidelberg, Im Neuenheimer Feld 410, Heidelberg, D-69120, Germany

11 Department of Dermatology and Allergology, Technical University of Munich, Biedersteiner Strasse 29, Munich, D-80802, Germany

12 Department of Rheumatology and Clinical Immunology, Kerckhoff Clinic, Justus-Liebig University, Benekestrasse 2, Bad Nauheim, D-61231, Germany

13 Department of Dermatology, Heinrich-Heine-University, Moorenstrasse 5, Düsseldorf, D-40225, Germany

14 Department of Dermatology, University of Münster, Von-Esmarch-Strasse 58, Münster, D-48149, Germany

15 Center of Rheumatology of Brandenburg, Johanniter Hospital in Fläming, Johanniterstrasse 1, Treuenbrietzen, D-14929, Germany

16 Department of Dermatology and Allergology, University of Ulm, Maienweg 12, Ulm, D-89081, Germany

17 Center for Rheumatology, Acura Hospital, Rotenbachtalstrasse 5, Baden-Baden, D-76530, Germany

18 Department of Dermatology, Venerology and Allergology, University of Leipzig, Philipp-Rosenthal-Strasse 23, Leipzig, 04103, Germany; present address: Department of Dermatology, University Hospital Erlangen, Ulmenweg 18, Erlangen, D-91054, Germany

19 Department of Dermatology and Allergology, Helios Klinikum, Heusnerstrasse 40, Wuppertal, D-42283, Germany

20 Department of Dermatology, Johannes-Wesling-Klinik, Hans-Nolte-Strasse 1, Minden, D-32429, Germany

21 Medical Clinic I, Hospital Cologne-Merheim, Ostmerheimer Strasse 200, Cologne, D-51109, Germany; present address: Medical Clinic 6, Marien-Hospital, Wanheimer Strasse 167a, Duisburg, D-47053, Germany

22 Department of Dermatology, Venerology and Allergology, University of Würzburg, Josef-Schneider-Strasse 2, Würzburg, D-97080, Germany; present address: Department of Dermatology, Venerology and Allergology, Georg-August-University, Von-Siebold-Strasse 3, Göttingen, D-37075, Germany

23 Hamburg Centre for Pediatric and Adolescence Rheumatology, Dehnhaide 120, Hamburg Eilbek, D-22081, Germany

24 Rheumaklinik Aachen, Burtscheider Markt 24, Aachen, D-52066, Germany

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Arthritis Research & Therapy 2011, 13:R172  doi:10.1186/ar3495

Published: 21 October 2011



In the present study, we analysed in detail nuclear autoantibodies and their associations in systemic sclerosis (SSc) patients included in the German Network for Systemic Scleroderma Registry.


Sera of 863 patients were analysed according to a standardised protocol including immunofluorescence, immunoprecipitation, line immunoassay and immunodiffusion.


Antinuclear antibodies (ANA) were detected in 94.2% of patients. In 81.6%, at least one of the autoantibodies highly associated with SSc or with overlap syndromes with scleroderma features was detected, that is, anti-centromere (35.9%) or anti-topoisomerase I (30.1%), followed in markedly lower frequency by antibodies to PM-Scl (4.9%), U1-ribonucleoprotein (U1-RNP) (4.8%), RNA polymerases (RNAPs) (3.8%), fibrillarin (1.4%), Ku (1.2%), aminoacyl-transfer RNA synthetases (0.5%), To (0.2%) and U11-RNP (0.1%). We found that the simultaneous presence of SSc-associated autoantibodies was rare (1.6%). Furthermore, additional autoantibodies were detected in 55.4% of the patients with SSc, of which anti-Ro/anti-La, anti-mitochondrial and anti-p25/p23 antibodies were most frequent. The coexistence of SSc-associated and other autoantibodies was common (43% of patients). SSc-associated autoantibodies disclosed characteristic associations with clinical features of patients, some of which were previously not acknowledged.


This study shows that five autoantigens (that is, centromere, topoisomerase I, PM-Scl, U1-RNP and RNAP) detected more than 95% of the known SSc-associated antibody responses in ANA-positive SSc patients and characterise around 79% of all SSc patients in a central European cohort. These data confirm and extend previous data underlining the central role of the determination of ANAs in defining the diagnosis, subset allocation and prognosis of SSc patients.

systemic sclerosis; scleroderma; autoantibodies; antinuclear antibodies