Artikel
Differential loss of diffusing capacity in SSc subsets – data from the German Network for Systemic Scleroderma
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Autoren
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Pia Moinzadeh
- Universitätsklinikum Köln, Klinik und Poliklinik für Dermatologie und Venerologie, Köln
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Elise Siegert
- Charité - Universitätsmedizin Berlin, Medizinische Klinik mit Schwerpunkt Rheumatologie und klinische Immunologie, Berlin
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Norbert Blank
- Universitätsklinikum Heidelberg, Medizinische Klinik V, Sektion Rheumatologie, Heidelberg
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Jörg Henes
- Universitätsklinik Tübingen Zentrum für Interdisziplinäre Rheumatologie INDIRA und Medizinische Klinik II, Rheumatologie, Tübingen
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Cord Sunderkötter
- Universitätsklinikum Münster, Westfälische Wilhelms-Universität, Klinik für Dermatologie und Venerologie, Münster
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Ina Kötter
- Asklepios Klinik Altona, Rheumatologie, Klinische Immunologie, Nephrologie, Hamburg
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Marc Schmalzing
- Universitätsklinikum Würzburg, Medizinische Klinik und Poliklinik II, Schwerpunkt Rheumatologie und klinische Immunologie, Würzburg
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Laura Susok
- Universitätsklinikum der Ruhr-Universität Bochum, Dermatologie, Bochum
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Margitta Worm
- Charite - Universitätsmedizin Berlin, Dermatologie, Berlin
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Claudia Günther
- Universitätsklinikum Carl Gustav Carus, Klinik und Poliklinik für Dermatologie, Dresden
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Elisabeth Aberer
- Medizinische Universität Graz, Dermatologie, Graz, Österreich
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Tim Schmeiser
- Krankenhaus St. Josef Wuppertal, Klinik für Rheumatologie, Osteologie und Immunologie, Wuppertal
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Gabriela Riemekasten
- Universität zu Lübeck, Klinik für Rheumatologie und Klinische Immunologie, Lübeck
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Thomas Krieg
- Universitätsklinikum Köln, Klinik und Poliklinik für Dermatologie und Venerologie, Köln
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Alexander Kreuter
- HELIOS Privatklinik Oberhausen, Dermatologie, Venerologie und Allergologie, Oberhausen
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Gabriele Zeidler
- Johanniter-Krankenhaus im Fläming Treuenbrietzen GmbH, Treuenbrietzen
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Aaron Juche
- Immanuel Krankenhaus Berlin-Buch, Rheumatologie, Berlin
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Ulf Müller-Ladner
- Justus-Liebig-Universität Gießen, Kerckhoff-Klinik GmbH, Rheumatologie u. klinische Immunologie, Osteologie, Physikalische Therapie, Bad Nauheim
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Noemi Gäbelein-Wissing
- HELIOS Universitätsklinikum Wuppertal, Dermatologie, Wuppertal
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Kathrin Kuhr
- Institut für medizinische Statistik, Informatik und Epidemiologie, Universität zu Köln, Köln
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Nico Hunzelmann
- Universitätsklinikum Köln, Klinik und Poliklinik für Dermatologie und Venerologie, Köln
| Veröffentlicht: | 4. September 2017 |
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Gliederung
Text
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%.
Conclusion: 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.
