The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement.
The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement.
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DOI：
10.1093/rheumatology/ken179
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发表时间：
2008-08
期刊：
影响因子：
--
通讯作者：
Registry of the German Network for Systemic Scleroderma
中科院分区：
文献类型：
--
作者：
Hunzelmann N;Genth E;Krieg T;Lehmacher W;Melchers I;Meurer M;Moinzadeh P;Müller-Ladner U;Pfeiffer C;Riemekasten G;Schulze-Lohoff E;Sunderkoetter C;Weber M;Worm M;Klaus P;Rubbert A;Steinbrink K;Grundt B;Hein R;Scharffetter-Kochanek K;Hinrichs R;Walker K;Szeimies RM;Karrer S;Müller A;Seitz C;Schmidt E;Lehmann P;Foeldvári I;Reichenberger F;Gross WL;Kuhn A;Haust M;Reich K;Böhm M;Saar P;Fierlbeck G;Kötter I;Lorenz HM;Blank N;Gräfenstein K;Juche A;Aberer E;Bali G;Fiehn C;Stadler R;Bartels V;Registry of the German Network for Systemic Scleroderma
客观的。系统性硬化症（SSc）是一种罕见的异质性疾病，影响不同的器官，因此需要跨学科的诊断和治疗管理。为了改善对具有不同疾病表现的患者的检测和随访，在参与 SSc 患者护理的不同亚专业的贡献下，建立了一个跨学科登记处。方法。开发了一份调查问卷来收集一组核心临床数据以确定当前的疾病状态。患者被分为五个描述性疾病亚组，即 lcSSc、dcSSc、SSc 正弦硬皮病、重叠综合征和具有硬皮病特征的 UCTD。结果。在 1483 名患者中，45.5% 的患者患有 lcSSc，32.7% 的患者患有 dcSSc。 10.9% 的患者被诊断出重叠综合征，而 8.8% 的患者为未分化型。 1.5% 的患者患有 SSc 正弦硬皮病。不同亚群之间的器官受累情况明显不同；例如，肺纤维化在 dcSSc (56.1%) 中的发生率明显高于重叠综合征 (30.6%) 或 lcSSc (20.8%)。与 lcSSc (14.9%)、重叠综合征 (8.2%) 和未分化疾病 (4.1%) 相比，肺动脉高压在 dcSSc (18.5%) 中更为常见。肌肉骨骼受累是重叠综合征的典型特征（67.6%）。 17.2% 的患者有风湿性疾病家族史，且与疾病早期发病相关（P < 0.005）。结论。在这个全国范围的登记册中，将具有 SSc 特征的疾病表现的患者描述性分类为五组，从而可以包括更广泛的具有 SSc 特征的患者。
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.
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影响因子：
5.2
作者：
Alarcón, GS
通讯作者：
Alarcón, GS
影响因子：
1.6
作者：
Ferri, C;Valentini, G;Tirri, G
通讯作者：
Tirri, G
影响因子：
--
作者：
MARICQ, HR;WEINRICH, MC;ROSAL, EJ
通讯作者：
ROSAL, EJ
影响因子：
--
作者：
Steen, VD;Oddis, CV;Medsger, TA
通讯作者：
Medsger, TA
影响因子：
--
作者：
Sunderkotter, C.;Riernelkasten, G.
通讯作者：
Riernelkasten, G.