Artikel
Undifferentiated SSc, clinical manifestations and disease progression of 282 patients into definite SSc subsets – data of 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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Norbert Blank
- Universitätsklinikum Heidelberg, Medizinische Klinik V, Sektion Rheumatologie, Heidelberg
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Elise Siegert
- Charité - Universitätsmedizin Berlin, Medizinische Klinik mit Schwerpunkt Rheumatologie und klinische Immunologie, Berlin
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Jörg Henes
- Universitätsklinikum Tübingen, Innere Medizin II - Onkologie, Hämatologie, klinische Immunologie, Rheumatologie und Pulmologie, Tübingen
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Laura Susok
- Universitätsklinikum der Ruhr-Universität Bochum, Dermatologie, Bochum
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Jörg H. W. Distler
- Universitätsklinikum Erlangen, Medizinische Klinik 3, Rheumatologie und Immunologie, Erlangen
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Aaron Juche
- Immanuel Krankenhaus Berlin-Buch, Rheumatologie, Berlin
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Margitta Worm
- Charite - Universitätsmedizin Berlin, Dermatologie, Berlin
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Hannah Clara Ahrens
- Charité - Universitätsmedizin Berlin, Dermatologie, Berlin
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Tim Schmeiser
- Krankenhaus St. Josef Wuppertal, Klinik für Rheumatologie, Osteologie und Immunologie, Wuppertal
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Gabriele Zeidler
- Johanniter-Krankenhaus im Fläming Treuenbrietzen GmbH, Treuenbrietzen
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Noemi Gäbelein-Wissing
- HELIOS Universitätsklinikum Wuppertal, Dermatologie, Wuppertal
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Thomas Krieg
- Universitätsklinikum Köln, Klinik und Poliklinik für Dermatologie und Venerologie, Köln
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Cord Sunderkötter
- Universitätsklinikum Münster, Westfälische Wilhelms-Universität, Klinik für Dermatologie und Venerologie, Münster
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Alexander Kreuter
- HELIOS Privatklinik Oberhausen, Dermatologie, Venerologie und Allergologie, Oberhausen
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Miklos Sardy
- Ludwig-Maximilians-Universität München, Klinik und Poliklinik für Dermatologie und Allergologie, München
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Gabriela Riemekasten
- Klinik für Rheumatologie, Universität zu Lübeck, Lübeck
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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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Marc Frerix
- Justus-Liebig-Universität Gießen, Kerckhoff-Klinik GmbH, Rheumatologie u. klinische Immunologie, Osteologie, Physikalische Therapie, Bad Nauheim
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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: | 29. August 2016 |
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Gliederung
Text
Background: Systemic sclerosis (SSc) is a heterogeneous multisystem connective tissue disease, usually subdivided into the limited (lcSSc) and diffuse cutaneous SSc (dcSSc) by LeRoy and overlap syndromes. However, some patients present with symptoms suggestive of but not conclusive for a diagnosis of definite SSc. These patients have been referred to as very early or undifferentiated SSc subset (uSSc). It is defined as RP and at least one concomitant feature of SSc and/or detectable SSc-specific autoantibodies in SSc patients without fulfilling neither LeRoy nor ACR/EULAR SSc diagnosis criteria.
Methods: Up to date, more than 3400 patients have been registered within the German network for systemic scleroderma. All available follow-up visits of patients suffering from uSSc at initial patient registration were analysed to determine whether clinical features change over time and patients develop definite SSc.
Results: Among 3473 registered patients, 8.1% (282/3473) were diagnosed with uSSc at time of inclusion (mean age 59.9±1.5 years). A meaningful difference was detected as lcSSc patients were approximately 5 years and dcSSc 10 years younger than uSSc patients at time of diagnosis. Antinuclear antibodies (ANA) were positive in 77.7% (40.2% anti-centromer (ACA), 12.8% anti-topoisomerase (ATA) and 47% other SSc-specific antibodies).
34.0% of uSSc patients suffered from sicca symptoms, followed by 31.2% showing gastrointestinal, 30.5% musculoskeletal involvement, 14.5% lung fibrosis, 6.0% heart involvement and 5.0% pulmonary arterial hypertension.
Musculoskeletal and heart involvement were found equally in patients with uSSc and lcSSc, but significantly more often in patients with dcSSc. Lung fibrosis, pulmonary arterial hypertension and gastrointestinal involvement occurred significantly less often in patients with uSSc compared to lcSSc and dcSSc. After a mean follow-up time of nearly 4 years, a progression into lcSSc was observed in 16.3%, 4.6% converted into dcSSc and 3.2% were classified as overlap syndromes, while the majority of the patients remained within the subset uSSc (76.6%).
Conclusion: Patients with uSSc have been significantly older than other subsets at time of diagnosis (i.e. 5-10 years). During follow-up progression into a limited, diffuse or SSc overlap syndrome subset was observed in every fourth uSSc patient, however, the majority did not develop definite SSc.
