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.
Hunzelmann
N
N
Department of Dermatology and Venerology, University of Cologne, Kerpener Str. 62, 50924 Cologne, Germany. Sklerodermie-Netzwerk@uk-koeln.de
Genth
E
E
Krieg
T
T
Lehmacher
W
W
Melchers
I
I
Meurer
M
M
Moinzadeh
P
P
Müller-Ladner
U
U
Pfeiffer
C
C
Riemekasten
G
G
Schulze-Lohoff
E
E
Sunderkoetter
C
C
Weber
M
M
Worm
M
M
Klaus
P
P
Rubbert
A
A
Steinbrink
K
K
Grundt
B
B
Hein
R
R
Scharffetter-Kochanek
K
K
Hinrichs
R
R
Walker
K
K
Szeimies
R-M
RM
Karrer
S
S
Müller
A
A
Seitz
C
C
Schmidt
E
E
Lehmann
P
P
Foeldvári
I
I
Reichenberger
F
F
Gross
W L
WL
Kuhn
A
A
Haust
M
M
Reich
K
K
Böhm
M
M
Saar
P
P
Fierlbeck
G
G
Kötter
I
I
Lorenz
H-M
HM
Blank
N
N
Gräfenstein
K
K
Juche
A
A
Aberer
E
E
Bali
G
G
Fiehn
C
C
Stadler
R
R
Bartels
V
V
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