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Extended report
Disease progression in systemic sclerosis-overlap syndrome is significantly different from limited and diffuse cutaneous systemic sclerosis
  1. Pia Moinzadeh1,
  2. Elisabeth Aberer2,
  3. Keihan Ahmadi-Simab3,
  4. Norbert Blank4,
  5. Joerg H W Distler5,
  6. Gerhard Fierlbeck6,
  7. Ekkehard Genth7,
  8. Claudia Guenther8,
  9. Ruediger Hein9,
  10. Joerg Henes10,
  11. Lena Herich11,
  12. Ilka Herrgott12,
  13. Ina Koetter13,
  14. Alexander Kreuter14,
  15. Thomas Krieg1,
  16. Kathrin Kuhr11,
  17. Hanns-Martin Lorenz15,
  18. Florian Meier16,
  19. Inga Melchers17,
  20. Hartwig Mensing18,
  21. Ulf Mueller-Ladner16,
  22. Christiane Pfeiffer19,
  23. Gabriela Riemekasten20,
  24. Miklós Sárdy21,
  25. Marc Schmalzing10,
  26. Cord Sunderkoetter12,
  27. Laura Susok22,
  28. Ingo H Tarner16,
  29. Peter Vaith23,
  30. Margitta Worm24,
  31. Gottfried Wozel8,
  32. Gabriele Zeidler25,
  33. Nicolas Hunzelmann1,
  34. and all participating DNSS centers
  1. 1Department of Dermatology, Cologne University Hospital, Cologne, Germany
  2. 2Department of Dermatology, Medical University of Graz, Graz, Germany
  3. 3Department of Rheumatology, Asklepios Clinic Altona, Hamburg, Germany
  4. 4Department of Internal Medicine, Division of Rheumatology, University of Heidelberg, Heidelberg, Germany
  5. 5Department of Rheumatology, University of Erlangen, Erlangen, Germany
  6. 6Department of Dermatology, University of Tuebingen, Aachen, Germany
  7. 7Department of Rheumatology, Clinic of Rheumatology of Aachen, Aachen, Germany
  8. 8Department of Dermatology, University-Hospital Carl Gustav Carus, Dresden, Germany
  9. 9Department of Dermatology, Munich University of Technology, Munich, Germany
  10. 10Department of Rheumatology, University of Tuebingen, Tuebingen, Germany
  11. 11Institute of Medical Statistics, Informatics and Epidemiology, University of Cologne, Cologne, Germany
  12. 12Department of Dermatology and Venereology, University of Muenster, Muenster, Germany
  13. 13Department of Internal Medicine and Nephrology (Centre for interdisciplinary Rheumatology), Robert-Bosch-Hospital, Stuttgart, Germany
  14. 14Department of Dermatology, Helios Clinic Oberhausen, Oberhausen, Germany
  15. 15Department of Haemato-Oncology and Rheumatology, University of Heidelberg, Heidelberg, Germany
  16. 16Department of Rheumatology and Clinical Immunology, Kerckhoff Clinic, Bad Nauheim, Germany
  17. 17Clinical Research Unit for Rheumatology, University Medical Center Freiburg, Freiburg, Germany
  18. 18Clinic for Dermatology, Hamburg Alstertal, Hamburg, Germany
  19. 19Department of Dermatology, Ulm University Hospital, Ulm, Germany
  20. 20Department of Rheumatology and Clinical Immunology, University of Berlin, Charité, Germany
  21. 21Department of Dermatology and Allergology, Ludwig Maximilian University, Munich, Germany
  22. 22Department of Dermatology and Venereology, Ruhr University Bochum, Bochum, Germany
  23. 23Department of Rheumatology and Clinical Immunology, University Medical Center Freiburg, Freiburg, Germany
  24. 24Department of Dermatology and Venerology, University of Berlin, Charité, Berlin, Germany
  25. 25Department of Rheumatology, Johanniter-Hospital, Treuenbrietzen, Germany
  1. Correspondence to Dr Pia Moinzadeh, Department of Dermatology and Venereology, University of Cologne, Kerpener Str. 62, Köln, Cologne 50937, Germany; pia.moinzadeh{at}uk-koeln.de

Abstract

Background Systemic sclerosis (SSc)-overlap syndromes are a very heterogeneous and remarkable subgroup of SSc-patients, who present at least two connective tissue diseases (CTD) at the same time, usually with a specific autoantibody status.

Objectives To determine whether patients, classified as overlap syndromes, show a disease course different from patients with limited SSc (lcSSc) or diffuse cutaneous SSc (dcSSc).

Methods The data of 3240 prospectively included patients, registered in the database of the German Network for Systemic Scleroderma and followed between 2003 and 2013, were analysed.

Results Among 3240 registered patients, 10% were diagnosed as SSc-overlap syndrome. Of these, 82.5% were female. SSc-overlap patients had a mean age of 48±1.2 years and carried significantly more often ‘other antibodies’ (68.0%; p<0.0001), including anti-U1RNP, -PmScl, -Ro, -La, as well as anti-Jo-1 and -Ku antibodies.

These patients developed musculoskeletal involvement earlier and more frequently (62.5%) than patients diagnosed as lcSSc (32.2%) or dcSSc (43.3%) (p<0.0001). The onset of lung fibrosis and heart involvement in SSc-overlap patients was significantly earlier than in patients with lcSSc and occurred later than in patients with dcSSc. Oesophagus, kidney and PH progression was similar to lcSSc patients, whereas dcSSc patients had a significantly earlier onset.

Conclusions These data support the concept that SSc-overlap syndromes should be regarded as a separate SSc subset, distinct from lcSSc and dcSSc, due to a different progression of the disease, different proportional distribution of specific autoantibodies, and of different organ involvement.

  • Autoimmune Diseases
  • Epidemiology
  • Systemic Sclerosis

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