The Heart in Scleroderma

Scleroderma is one of the collagen diseases, namely one that attacks primarily the mesenchymal collagen fibres of connective tissue. Those collagen fibres already present usually become thickened, but also new ones are formed. The effects of the disease are therefore not confined to the skin, as was thought originally, but are widespread throughout the body, and the term scleroderma is thus a misnomer. In addition to the skin, the main structures to be affected clinically are skeletal muscle, the alimentary system, especially the cesophagus, the lungs, kidneys, endocrine glands, bones, and joints, and the heart. The disease is prone to remission and relapse, but ultimately proves fatal whether it pursues a fulminating course of a few months or even weeks