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April 30, 1973

Section 6.— Connective Tissue Diseases

JAMA. 1973;224(Suppl_5):681-687. doi:10.1001/jama.1973.03220190021009

General Considerations.—  The diseases of connective tissue can be divided into a group of relatively uncommon, genetically determined disorders which affect the primary structure of this tissue (see Section 53: Heritable Disorders of Connective Tissue), and a number of acquired maladies in which the connective tissues serve as the locus of several more or less distinctive immunologic and inflammatory reactions. The latter conditions were originally designated as "diffuse collagen diseases" by Klemperer and his co-workers,1,2 who applied the term to certain "acute and chronic maladies which are characterized anatomically by generalized alterations of the connective tissue, particularly by abnormalities of its extracellular components." The idea that the histopathologic changes found in rheumatic fever and rheumatoid arthritis reflected a disturbance in the connective tissue system had first been proposed by Klinge.3 The connective tissues were earlier known as the collagen or collagenvascular system. Now that the term collagen has