The registry of the german network for systemic scleroderma: Frequency of disease subsets and patterns of organ involvement

N. Hunzelmann, E. Genth, T. Krieg, W. Lehmacher, I. Melchers, M. Meurer, P. Moinzadeh, U. Müller-Ladner, C. Pfeiffer, G. Riemekasten, E. Schulze-Lohoff, C. Sunderkoetter, M. Weber, M. Worm, P. Klaus, A. Rubbert, K. Steinbrink, B. Grundt, R. Hein, K. Scharffetter-KochanekR. Hinrichs, K. Walker, R. M. Szeimies, S. Karrer, A. Müller, C. Seitz, E. Schmidt, P. Lehmann, I. Foeldvári, F. Reichenberger, W. L. Gross, A. Kuhn, M. Haust, K. Reich, M. Böhm, P. Saar, G. Fierlbeck, I. Kötter, H. M. Lorenz, N. Blank, K. Gräfenstein, A. Juche, E. Aberer, G. Bali, C. Fiehn, R. Stadler, V. Bartels

179 Citations (Scopus)


Objective: Systemic sclerosis (SSc) is a rare, heterogeneous disease, which affects different organs and therefore requires interdisciplinary diagnostic and therapeutic management. To improve the detection and follow-up of patients presenting with different disease manifestations, an interdisciplinary registry was founded with contributions from different subspecialties involved in the care of patients with SSc. Methods: A questionnaire was developed to collect a core set of clinical data to determine the current disease status. Patients were grouped into five descriptive disease subsets, i.e. lcSSc, dcSSc, SSc sine scleroderma, overlap-syndrome and UCTD with scleroderma features. Results: Of the 1483 patients, 45.5% of patients had lcSSc and 32.7% dcSSc. Overlap syndrome was diagnosed in 10.9% of patients, while 8.8% had an undifferentiated form. SSc sine scleroderma was present in 1.5% of patients. Organ involvement was markedly different between subsets; pulmonary fibrosis for instance was significantly more frequent in dcSSc (56.1%) than in overlap syndrome (30.6%) or lcSSc (20.8%). Pulmonary hypertension was more common in dcSSc (18.5%) compared with lcSSc (14.9%), overlap syndrome (8.2%) and undifferentiated disease (4.1%). Musculoskeletal involvement was typical for overlap syndromes (67.6%). A family history of rheumatic disease was reported in 17.2% of patients and was associated with early disease onset (P < 0.005). Conclusion: In this nationwide register, a descriptive classification of patients with disease manifestations characteristic of SSc in five groups allows to include a broader spectrum of patients with features of SSc.

Original languageEnglish
Issue number8
Pages (from-to)1185-1192
Number of pages8
Publication statusPublished - 08.2008

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)


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