Volume 69 Number 4 September 2002	back to contents

Apoptosis: A Case Where Too Much or Too Little Can Lead to Autoimmunity	208-219
Tamara Grodzicky, M.D., F.R.C.P.C.1, and Keith B. Elkon, M.D.2

From the ¹Department of Medicine, Rheumatology Division and ²Research Division, Hospital for Special Surgery-Weill Medical College of Cornell University, New York, NY.

Address correspondence to Keith B. Elkon, M.D., Division of Rheumatology, Box 356428, University of Washington, 1959 NE Pacific Street, Seattle, WA 98195-6428.

Adapted from a Grand Rounds presentation by Dr. Elkon to the Department of Medicine, Mount Sinai School of Medicine, New York, NY on April 4, 2000 and updated as of August 2001.

This work was supported by grants from the Royal College of Physicians and Surgeons of Canada-Medical Research Council of Canada-Novartis Fellowship Award (TG) and the National Institutes of Health (KBE).

ABSTRACT

Apoptosis is a physiological process of cell death that normally occurs when cells are damaged or no longer needed. One of its major roles is the maintenance of peripheral immune tolerance, by eliminating activated T and B cells beyond the course of an infection, and thus terminating immune responses. When apoptosis becomes dysfunctional, either being “too much” or “too little,” a variety of different disease states may be triggered. For example, insufficient apoptosis of activated immune cells is the basis of the Canale Smith Syndrome /ALPS, whereas excessive apoptosis of ~ islet cells of the pancreas is involved in the pathogenesis of autoimmune diabetes mellitus. In this review, we explain the fundamental aspects and molecular mechanisms of apoptosis and their relevance to several important human autoimmune diseases.

KEYWORDS

Apoptosis, autoimmunity, diabetes mellitus, Canale-Smith Syndrome/ALPS, acute lymphoproliferative syndrome, multiple sclerosis, systemic lupus erythematosus, rheumatoid arthritis.

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