Systemic sclerosis - A systematic overview Part 1-Disease characteristics and classification, pathophysiologic concepts, and recommendations for diagnosis and surveillance

被引:29
作者
Klein-Weigel, P. [3 ]
Opitz, C. [1 ]
Riemekasten, G. [2 ]
机构
[1] DRK Kliniken Berlin, Innere Med Klin, Berlin, Germany
[2] Charite, Med Klin Schwerpunkt Rheumatol & Klin Immunol, D-13353 Berlin, Germany
[3] DRK Kliniken Berlin, Klin Innere Med Schwerpunkt Angiol & Kardiovaskul, Berlin, Germany
关键词
Scleroderma disorder; systemic sclerosis; Raynaud's phenomenon; digital ulcer; pulmonary arterial hypertension; capillary microscopy; CONNECTIVE-TISSUE DISEASE; INTERSTITIAL LUNG-DISEASE; PRIMARY BILIARY-CIRRHOSIS; SCLERODERMA RENAL CRISIS; RAYNAUDS-PHENOMENON; SJOGRENS-SYNDROME; PULMONARY-HYPERTENSION; GROWTH-FACTOR; STIMULATORY AUTOANTIBODIES; NAILFOLD CAPILLAROSCOPY;
D O I
10.1024/0301-1526/a000065
中图分类号
R6 [外科学];
学科分类号
1002 ; 100210 ;
摘要
Due to its high association with Raynaud's phenomenon systemic sclerosis (SSc) is probably the most common connective tissue disease seen by vascular specialists. In part 1 of our systematic overview we summarize classification concepts of scleroderma disorders, the epidemiologic and genetic burden, the complex pathophysiologic background, and the clinical features and the stage-dependent capillary microscopic features of SSc. Furthermore, we address the diagnostic recommendations propagated by the German Network for Systemic Sclerosis and the Task Force for Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology, the European Respiratory Society, and the International Society of Heart and Lung Transplantation
引用
收藏
页码:6 / 19
页数:14
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