Rheumatology Advance Access originally published online on May 31, 2008
Rheumatology 2008 47(8):1185-1192; doi:10.1093/rheumatology/ken179
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The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement
1Department of Dermatology and Venerology, University of Cologne, Cologne, 2Department of Rheumatology, University of Aachen, Aachen, 3Institute of Biostatistics, Informatics and Epidemiology, University of Cologne, Cologne, 4Clinical Research Unit for Rheumatology, University Medical Center Freiburg, Freiburg ,5Department of Dermatology, Dresden University Hospital, Dresden, 6Department of Rheumatology and Clinical Immunology, Kerckhoff Clinic, Bad Nauheim, 7Rheumatology and Clinical Immunology, Charité, Berlin, 8Hospital Cologne-Merheim, Medical Clinic I, 9Department of Dermatology, University of Münster, Münster, 10Department of Dermatology, Venerology and Allergology, University Hospital Charité, Berlin, 11Department of Rheumatology, University of Cologne, Cologne, 12Department of Dermatology, University of Mainz, Mainz, 13Department of Dermatology and Allergology, University of Munich, Munich, 14Department of Dermatology and Allergology, University of Ulm, Ulm, 15Department of Dermatology, 16Department of Internal Medicine, University of Regensburg, Regensburg, 17Department of Internal Medicine, University of Regensburg, Regensburg, 18Department of Dermatology, Allergology and Environmental Medicine, Private University Witten-Herdecke, HELIOS Klinikum Wuppertal, Wuppertal, 19Hospital Eilbek, Hamburg, 20Department of Internal Medicine, University of Giessen, Giessen, 21Clinic for Rheumatology, Bad Bramstedt, 22Department of Dermatology, University of Düsseldorf, Düsseldorf, 23Department of Dermatology and Venerology,Georg-August-University of Göttingen, Göttingen, 24Department of Dermatology, University of Tübingen, Tübingen, 25Department of Internal Medicine, University of Tübingen, Tübingen, 26Department of Internal Medicine, University of Heidelberg, Heidelberg, 27Johanniter Hospital in Fläming gGmbH, Center of Rheumatology of Brandenburg, Germany, 28Department of Dermatology and Venerology, University of Graz, Graz, Austria, 29Department of Internal Medicine, Center of Rheumatology, Baden-Baden and 30Department of Dermatology, Clinic of Minden, Minden, Germany.
Correspondence to: N. Hunzelmann, Department of Dermatology and Venerology, University of Cologne, Kerpener Str. 62, 50924 Cologne, Germany. E-mail: Sklerodermie-Netzwerk{at}uk-koeln.de
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Abstract |
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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.
KEY WORDS: Systemic sclerosis, Scleroderma, Connective tissue disease, Overlap syndrome, Undifferentiated disease
Submitted 14 November 2007;
revised version accepted 2 April 2008.
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