Hunzelmann, N. and Genth, E. and Krieg, T. and Lehmacher, W. and Melchers, I. and Meurer, M. and Moinzadeh, P. and Mueller-Ladner, U. and Pfeiffer, C. and Riemekasten, G. and Schulze-Lohoff, E. and Sunderkoetter, C. and Weber, M. and Worm, M. and Klaus, P. and Rubbert, A. and Steinbrink, K. and Grundt, B. and Hein, R. and Scharffetter-Kochanek, K. and Hinrichs, R. and Walker, K. and Szeimies, R. -M. and Karrer, S. and Mueller, A. and Seitz, C. and Schmidt, E. and Lehmann, P. and Foeldvari, I. and Reichenberger, F. and Gross, W. L. and Kuhn, A. and Haust, M. and Reich, K. and Boehm, M. and Saar, P. and Fierlbeck, G. and Koetter, I. and Lorenz, H. -M. and Blank, N. and Graefenstein, K. and Juche, A. and Aberer, E. and Bali, G. and Fiehn, C. and Stadler, R. and Bartels, V. (2008) The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement. RHEUMATOLOGY, 47 (8). pp. 1185-1192. ISSN 1462-0324, 1462-0332
Full text not available from this repository. (Request a copy)Abstract
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.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | SCLEROSIS SINE SCLERODERMA; CONNECTIVE-TISSUE DISEASES; SEROLOGIC FEATURES; EPIDEMIOLOGY; CLASSIFICATION; SURVIVAL; POPULATION; PREVALENCE; THICKNESS; CRITERIA; systemic sclerosis; scleroderma; connective tissue disease; overlap syndrome; undifferentiated disease |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Dermatologie und Venerologie Medicine > Lehrstuhl für Innere Medizin I |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 28 Oct 2020 06:26 |
| Last Modified: | 28 Oct 2020 06:26 |
| URI: | https://pred.uni-regensburg.de/id/eprint/30567 |
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