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


CONCLUSIONS
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.
Department of Dermatology and Venerology, University of Cologne, Kerpener Str. 62, 50924 Cologne, Germany. Sklerodermie-Netzwerk@uk-koeln.de
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Hunzelmann
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Genth
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Krieg
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Lehmacher
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Melchers
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Meurer
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Moinzadeh
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Müller-Ladner
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Pfeiffer
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Riemekasten
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Schulze-Lohoff
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Sunderkoetter
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Weber
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Worm
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Klaus
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Rubbert
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Steinbrink
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Grundt
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Hein
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Scharffetter-Kochanek
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Hinrichs
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Walker
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Szeimies
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Karrer
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Müller
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Seitz
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Schmidt
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Lehmann
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Foeldvári
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Reichenberger
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Gross
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Kuhn
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Haust
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Reich
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Böhm
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Saar
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Fierlbeck
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Kötter
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Lorenz
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Blank
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Gräfenstein
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Juche
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Aberer
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Bali
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Fiehn
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Stadler
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Bartels
D000328:Adult; D017677:Age Distribution; D017668:Age of Onset; D000368:Aged; D003430:Cross-Sectional Studies; D005260:Female; D005858:Germany / Q000453:epidemiology; D006801:Humans; D008297:Male; D008511:Medicine; D008875:Middle Aged; D012042:Registries; D045743:Scleroderma, Diffuse / Q000453:epidemiology / Q000473:pathology; D045745:Scleroderma, Limited / Q000453:epidemiology / Q000473:pathology; D012595:Scleroderma, Systemic / Q000145:classification / Q000453:epidemiology* / Q000473:pathology; D013038:Specialization