Collagen Vascular Diseases and Disorders of Connective Tissue

The collagen vascular diseases, also referred to as connective tissue diseases, are a diverse group of systemic inflammatory disorders thought to be immunologically mediated. The concept of collagen vascular disease began to take shape in the 1930s, when it was recognized that rheumatic fever and rheumatoid arthritis can affect connective tissues throughout the body.1,2 During the following decade, as conditions such as systemic lupus erythematosus (SLE) and scleroderma came to be viewed as systemic diseases of connective tissue, the terms diffuse connective disease and diffuse collagen disease were proposed.3,4 During the same period, the designation of diffuse vascular disease was proposed for diseases such as scleroderma, polymyositis, SLE, and polyarteritis nodosa, which featured widespread vascular involvement.5 With the realization that many of these entities can exhibit both systemic connective tissue manifestations and vascular abnormalities, the unifying designation of collagen vascular disease was introduced.6