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AB0181 Diffusing capacity and clinical characteristics of patients with systemic sclerosis – data from the german network for systemic sclerosis
  1. P Moinzadeh1,
  2. N Blank2,
  3. E Siegert3,
  4. J Henes4,
  5. M Worm5,
  6. C Sunderkoetter6,
  7. M Schmalzing7,
  8. A Kreuter8,
  9. C Gunther9,
  10. L Susok10,
  11. G Zeidler11,
  12. I Koetter12,
  13. U Mueller-Ladner13,
  14. T Krieg1,
  15. A Juche14,
  16. T Schmeiser15,
  17. G Riemekasten16,
  18. E Aberer17,
  19. N Gaebelein-Wissing18,
  20. JHW Distler19,
  21. M Sárdy20,
  22. C Pfeiffer21,
  23. K Kuhr22,
  24. N Hunzelmann1,
  25. F Bonella23,
  26. M Kreuter24
  1. 1Dermatology, University Hospital Cologne, Cologne
  2. 2Rheumatology, University Hospital Heidelberg, Heidelberg
  3. 3Rheumatology, Charité Universitätsmedizin Berlin, Berlin
  4. 4Rheumatology, University Hospital Tuebingen, Tuebingen
  5. 5Dermatology, Charité Universitätsmedizin Berlin, Berlin
  6. 6Dermatology, University Hospital Muenster, Muenster
  7. 7Rheumatology, University Hospital Wuerzburg, Wuerzburg
  8. 8Dermatology, HELIOS St. Elisabeth Klinik Oberhausen, Oberhausen
  9. 9Dermatology, University Hospital Carl Gustav Carus, Dresden
  10. 10Dermatology, St. Josef Hospital Bochum, Bochum
  11. 11Rheumatology, Johanniter-Krankenhaus im Fläming Treuenbrietzen, Treuenbrietzen
  12. 12Rheumatology, Asklepios Klinik Altona, Hamburg
  13. 13Rheumatology, Justus Liebig University Giessen, Kerckhoff Clinic, Bad Nauheim
  14. 14Immanuel Krankenhaus Berlin-Buch, Berlin
  15. 15Rheumatology, Krankenhaus St. Josef, Wuppertal
  16. 16Rheumatology, University Medical Center-UKSH, Luebeck, Germany
  17. 17Dermatology, Medical University of Graz, Graz, Austria
  18. 18Dermatology, HELIOS University Hospital Wuppertal, Wuppertal
  19. 19Rheumatology, University Hospital Erlangen, Erlangen
  20. 20Dermatology, Ludwig Maximilians University Hospital, Munich
  21. 21Dermatology, University Medical Center Ulm, Ulm
  22. 22IMSIE, University of Cologne, Cologne
  23. 23Unit for interstitial lung diseases, Ruhrlandklinik University Hospital Essen, Essen
  24. 24Center for interstitial and rare lung diseases, Thoraxklinik, University Hospital Heidelberg, Heidelberg, Germany

Abstract

Background Lung involvement, i.e. interstitial lung disease (ILD) and pulmonary hypertension (PH), is common in patients with systemic sclerosis (SSc), significantly limiting quality of life and survival. Data on clinical correlations between lung function and clinical subsets of SSc are sparse.

Objectives To investigate the relationship of DLCO and clinical characteristics in patients SSc patients within the registry of the German Network for Systemic Scleroderma.

Methods Clinical data of the patient registry, currently including DLCO data of 1917 patients were evaluated. In total, these patients were clinically evaluated 5997 times (i.e., at the first visit and during follow-up visits). At the initial visit and during follow-up DLCO levels were correlated with clinical characteristics.

Results At initial presentation, 64% of the patients had DLCO levels <75% predicted. Impaired DLCO levels were observed in 74% of dcSSc patients, in 64% of SSc-Overlap patients and 57% of lcSSc patients (p<0.0001). Furthermore, male patients (62%), patients with PH (80%), ILD (80%), dyspnea (78%), and those with presence of anti-topoisomerase I antibodies (71%) exhibited significantly more often DLCO levels <75% (p<0.01). Patients suffering from dcSSc had the lowest DLCO levels (mean value, 62%), followed by patients with SSc-Overlap syndromes (mean value, 67%) and lcSSc patients (mean value, 70%) revealing significant differences between subsets. Long-term follow-up evaluation (mean follow up, 6.0 years) revealed that in comparison to lcSSc patients dcSSc patients (OR 2.1; p<0.0001; 95%>CI 1.7–2.5) and SSc-Overlap patients (OR, 1.55; p<0.0001; 95% CI, 1.2–2.0) had a significantly increased risk to a decrease in DLCO levels <75%.

Conclusions Impairment of pulmonary function as determined by diffusing capacity DLCO is more common and more pronounced in patients with dcSSc and SSc-Overlap Syndrome compared to lcSSc. DLCO may be useful for diagnosing and monitoring pulmonary involvement in SSc.

Disclosure of Interest None declared

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