The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement

• Published in: Rheumatology (Oxford, England) England), 2008-08, Vol.47 (8), p.1185-1192
• Main Authors: Hunzelmann, N., Genth, E., Krieg, T., Lehmacher, W., Melchers, I., Meurer, M., Moinzadeh, P., Müller-Ladner, U., Pfeiffer, C., Riemekasten, G., Schulze-Lohoff, E., Sunderkoetter, C., Weber, M., Worm, M., Klaus, P., Rubbert, A., Steinbrink, K., Grundt, B., Hein, R., Scharffetter-Kochanek, K., Hinrichs, R., Walker, K., Szeimies, R.-M., Karrer, S., Müller, A., Seitz, C., Schmidt, E., Lehmann, P., Foeldvári, I., Reichenberger, F., Gross, W.L., Kuhn, A., Haust, M., Reich, K., Böhm, M., Saar, P., Fierlbeck, G., Kötter, I., Lorenz, H.-M., Blank, N., Gräfenstein, K., Juche, A., Aberer, E., Bali, G., Fiehn, C., Stadler, R., Bartels, V.
• Format: Article
• Language: English
• Subjects: Adult; Age Distribution; Age of Onset; Aged; Biological and medical sciences; Clinical; Connective tissue disease; Cross-Sectional Studies; Diseases of the osteoarticular system; Female; Germany - epidemiology; Humans; Male; Medical sciences; Medicine; Middle Aged; Overlap syndrome; Registries; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Scleroderma; Scleroderma, Diffuse - epidemiology; Scleroderma, Diffuse - pathology; Scleroderma, Limited - epidemiology; Scleroderma, Limited - pathology; Scleroderma, Systemic - classification; Scleroderma, Systemic - epidemiology; Scleroderma, Systemic - pathology; Specialization; Systemic sclerosis; Undifferentiated disease
• ISSN: 1462-0324; 1462-0332
• DOI: 10.1093/rheumatology/ken179

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.