Connective tissue disease

Article By:

Stevens, Mary Betty Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland.

Inman, Robert D. Department of Immunology, University of Toronto, Toronto, Ontario, Canada.

Last reviewed:April 2019

DOI:https://doi.org/10.1036/1097-8542.157350

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• Connective tissue disease, published June 2014:Download PDF Get Adobe Acrobat Reader

Any of a group of diseases involving connective tissue as the pathological target. Connective tissue comprises one of the primary tissues of the body and is distinguished by an abundance of fibrillar and nonfibrillar extracellular components, which help to connect various structures of the body together. Connective tissue diseases (formerly termed collagen vascular diseases because collagen is one of the main protein components of connective tissue) are clinically and pathologically discrete, but have overlapping features. Specific types of connective tissue diseases include lupus erythematosus (Fig. 1), systemic vasculitis, scleroderma (and systemic sclerosis), polymyositis, and Sjögren's syndrome. Each of the diseases can involve multiple organ systems and is often coupled with various immunologic abnormalities. Often, the signs and symptoms of a variety of connective tissue diseases occur simultaneously. Under these circumstances, the condition is called mixed connective tissue disease. See also: Collagen; Connective tissue; Immunology; Tissue

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