Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Hunzelmann, N; Genth, E; Krieg, T; Lehmacher, W; Melchers, I; Meurer, M; Moinzadeh, P; Muller-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, RM; Karrer, S; Muller, A; Seitz, C; Schmidt, E; Lehmann, P; Foeldvari, I; Reichenberger, F; Gross, WL; Kuhn, A; Haust, M; Reich, K; Bohm, M; Saar, P; Fierlbeck, G; Kotter, I; Lorenz, HM; Blank, N; Grafenstein, K; Juche, A; Aberer, E; Bali, G; Fiehn, C; Stadler, R; Bartels, V.
The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement.
RHEUMATOLOGY. 2008; 47(8): 1185-1192.
Doi: 10.1093/rheumatology/ken179
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- Co-Autor*innen der Med Uni Graz
- Aberer Elisabeth
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- 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.
- Find related publications in this database (using NLM MeSH Indexing)
- Adult -
- Age Distribution -
- Age of Onset -
- Aged -
- Cross-Sectional Studies -
- Female -
- Germany - epidemiology
- Humans -
- Male -
- Middle Aged -
- Registries -
- Scleroderma, Diffuse - epidemiology
- Scleroderma, Limited - epidemiology
- Scleroderma, Systemic - classification
- Specialties, Medical -
- Find related publications in this database (Keywords)
- systemic sclerosis
- scleroderma
- connective tissue disease
- overlap syndrome
- undifferentiated disease