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

被引：208

作者：

Hunzelmann, N. ^{[1
]}

Genth, E. ^{[2
]}

Krieg, T. ^{[1
]}

Lehmacher, W. ^{[3
]}

Melchers, I. ^{[4
]}

Meurer, M. ^{[5
]}

Moinzadeh, P. ^{[1
]}

Mueller-Ladner, U. ^{[6
]}

Pfeiffer, C. ^{[5
]}

Riemekasten, G. ^{[7
]}

Schulze-Lohoff, E. ^{[8
]}

Sunderkoetter, C. ^{[9
]}

Weber, M. ^{[8
]}

Worm, M. ^{[10
]}

Klaus, P. ^{[10
]}

Rubbert, A. ^{[11
]}

Steinbrink, K. ^{[12
]}

Grundt, B. ^{[12
]}

Hein, R. ^{[13
]}

Scharffetter-Kochanek, K. ^{[14
]}

Hinrichs, R. ^{[14
]}

Walker, K. ^{[14
]}

Szeimies, R. -M. ^{[15
]}

Karrer, S. ^{[15
]}

Mueller, A. ^{[16
]}

Seitz, C. ^{[16
]}

Schmidt, E. ^{[16
]}

Lehmann, P. ^{[17
]}

Foeldvari, I. ^{[18
]}

Reichenberger, F. ^{[19
]}

Gross, W. L. ^{[20
]}

Kuhn, A. ^{[21
]}

Haust, M. ^{[21
]}

Reich, K. ^{[22
]}

Boehm, M.

Saar, P. ^{[16
]}

Fierlbeck, G. ^{[23
]}

Koetter, I. ^{[24
]}

Lorenz, H. -M. ^{[25
]}

Blank, N. ^{[25
]}

Graefenstein, K.

Juche, A.

Aberer, E. ^{[26
]}

Bali, G. ^{[26
]}

Fiehn, C. ^{[27
]}

Stadler, R. ^{[28
]}

Bartels, V. ^{[28
]}

机构：

[1] Univ Cologne, Dept Dermatol & Venerol, D-50924 Cologne, Germany

[2] Univ Aachen, Dept Rheumatol, D-5100 Aachen, Germany

[3] Univ Cologne, Inst Biostat Informat & Epidemiol, D-50924 Cologne, Germany

[4] Univ Med Ctr Freiburg, Clin Res Unit Rheumatol, Freiburg, Germany

[5] Dresden Univ Hosp, Dept Dermatol, Dresden, Germany

[6] Kerckhoff Clin, Dept Rheumatol & Clin Immunol, Bad Nauheim, Germany

[7] Charite, Berlin, Germany

[8] Hosp Cologne Merheim, Med Clin 1, Cologne, Germany

[9] Univ Munster, Dept Dermatol, D-4400 Munster, Germany

[10] Univ Hosp Charite, Dept Dermatol Venerol & Allergol, Berlin, Germany

[11] Univ Cologne, Dept Rheumatol, D-50924 Cologne, Germany

[12] Johannes Gutenberg Univ Mainz, Dept Dermatol, Mainz, Germany

[13] Univ Munich, Dept Dermatol & Allergol, Munich, Germany

[14] Univ Ulm, Dept Dermatol & Allergol, Ulm, Germany

[15] Univ Regensburg, Dept Dermatol, Regensburg, Germany

[16] Univ Regensburg, Dept Internal Med, Regensburg, Germany

[17] Private Univ Witten Herdecke, HELIOS Klinikum Wuppertal, Dept Dermatol Allergol & Environm Med, Wuppertal, Germany

[18] Hosp Eilbek, Hamburg, Germany

[19] Univ Giessen, Dept Internal Med, D-6300 Giessen, Germany

[20] Clin Rheumatol, Bad Bramstedt, Germany

[21] Univ Dusseldorf, Dept Dermatol, D-4000 Dusseldorf, Germany

[22] Univ Gottingen, Dept Dermatol & Venerol, Gottingen, Germany

[23] Univ Tubingen, Dept Dermatol, Tubingen, Germany

[24] Univ Tubingen, Dept Internal Med, D-7400 Tubingen, Germany

[25] Heidelberg Univ, Dept Internal Med, D-6900 Heidelberg, Germany

[26] Graz Univ, Dept Dermatol & Venerol, Graz, Austria

[27] Ctr Rheumatol, Dept Internal Med, Baden, Switzerland

[28] Clin Minden, Dept Dermatol, Minden, Germany

来源：

RHEUMATOLOGY | 2008年 / 47卷 / 08期

关键词：

systemic sclerosis; scleroderma; connective tissue disease; overlap syndrome; undifferentiated disease;

D O I：

10.1093/rheumatology/ken179

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：1185 / 1192

页数：8

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