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Large data bases and the projects arising from them have led to a much improved understanding of systemic sclerosis over the last decade. Serology has developed further so that more autoantibodies are available for routine testing. Capillary microscopy has become standard and relevant progress has also been made in therapy. Many diagnostic terms found in medical documentation do not adequately reflect this progress. The nomenclature is inconsistent and, therefore, confusing. The international classification of diseases (ICD) nomenclature is, from our point of view, also in need of improvement. This article aims to reestablish a common German language standard for systemic sclerosis, which reflects current knowledge and is suitable for implementation in the clinical routine.

The term Raynaud's phenomenon describes an abnormal vasospastic response to cold or emotional stress. It is a common condition with a prevalence of 3-5% of the population. Clinically, Raynaud's phenomenon manifests as sharply demarcated colour changes of the skin of the digits that is often accompanied by paraesthesia. Raynaud's phenomenon can be subdivided into primary, or idiopathic, and secondary forms, in the latter of which associated diseases or causes can be identified. The pathogenesis of the disease is incompletely understood. Pathologic changes have been observed primarily in vascular smooth muscle cells, endothelial cells and perineuronal microvasculature. Current therapeutic strategies include supportive treatments, topical therapeutic approaches and systemic medication. Drug therapies with proven efficacy include calcium channel blockers, prostacyclin analogues, fluoxetine, losartan and sildenafil.

In contrast to vasculogenesis, angiogenesis is defined as the formation of new vessels from preexisting ones. Physiologically, this multistep process occurs in adults during the reproductive cycle and during pregnancy, pathophysiologically it can be found in wound healing, inflammation and carcinogenesis. The underlying mechanisms are vasodilatation and increasing permeability, destabilization of vessel walls and degradation of extracellular matrix, followed by the proliferation and migration of endothelial cells. Migrated endothelial cells form vascular tubes at sites of ischemia and these tubes are finally stabilized by pericytes and smooth muscle cells. This process is controlled by a complex interaction of angiogenic and angiostatic factors. In contrast to carcinogenesis, the role of angiogenesis for the pathogenesis and therapy of rheumatic diseases is less understood. Two examples for pathologically disturbed angiogenesis, rheumatoid arthritis and systemic sclerosis, are discussed in this review with respect to therapeutic options.