We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement.
- Authors
N. Hunzelmann; E. Genth; T. Krieg; W. Lehmacher; I. Melchers; M. Meurer; P. Moinzadeh; U. Müller-Ladner; C. Pfeiffer; G. Riemekasten; E. Schulze-Lohoff; C. Sunderkoetter; M. Weber; M. Worm; P. Klaus; A. Rubbert; K. Steinbrink; B. Grundt; R. Hein; K. Scharffetter-Kochanek
- 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 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.
- Subjects
SCLERODERMA (Disease); SYSTEMIC scleroderma; PULMONARY fibrosis; PULMONARY hypertension
- Publication
Rheumatology, 2008, Vol 47, Issue 8, p1185
- ISSN
1462-0324
- Publication type
Article
- DOI
10.1093/rheumatology/ken179