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Rheumatology (Oxford). 2008 Aug;47(8):1185-92. doi: 10.1093/rheumatology/ken179. Epub 2008 May 31.

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

Author information

1
Department of Dermatology and Venerology, University of Cologne, Kerpener Str. 62, 50924 Cologne, Germany. Sklerodermie-Netzwerk@uk-koeln.de

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.

PMID:
18515867
PMCID:
PMC2468885
DOI:
10.1093/rheumatology/ken179
[Indexed for MEDLINE]
Free PMC Article
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