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Titel:

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

Dokumenttyp:
Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't; Article
Autor(en):
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, RM; Karrer, S; Müller, A; Seitz, C; Schmidt, E; Lehmann, P; Foeldvari, I; Reichenberger, F; Gross, WL; Kuhn, A; Haust, M; Reich, K; Böhm, M; Saar, P; Fierlbeck, G; Kotter, I; Lorenz, HM; Blank,...     »
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.
Zeitschriftentitel:
Rheumatology (Oxford)
Jahr:
2008
Band / Volume:
47
Heft / Issue:
8
Seitenangaben Beitrag:
1185-92
Sprache:
eng
Volltext / DOI:
doi:10.1093/rheumatology/ken179
PubMed:
http://view.ncbi.nlm.nih.gov/pubmed/18515867
Print-ISSN:
1462-0324
TUM Einrichtung:
Klinik und Poliklinik für Dermatologie und Allergologie
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