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Recent Advances in Thrombosis and Hemostasis 2008 pp 521–535Cite as

Etiopathology of the Antiphospholipid Syndrome

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Abstract

The antiphospholipid syndrome (APS) is an autoimmune disorder in which vascular thrombosis and pregnancy morbidity occur in patients with laboratory evidence of antiphospholipid antibodies (aPLs). Phospholipid-binding plasma proteins, β2-glycoprotein I (β2GPI) and prothrombin, are the dominant antigenic targets in APS. The aPLs affect the normal coagulation or anticoagulation reactions occurring on cell membranes and also interact with certain cells, altering the expression and secretion of procoagulant substances. Despite the clear association between aPLs and thrombotic events, the precise underlying disease mechanisms in APS remain unclear. Recently, great interest has focused on the cell membrane receptors and on the signal transduction pathways involved in the procoagulant cell activation mediated by aPLs. The roles of cell surface receptors for β2GPI, including those of the apolipoprotein E receptor 2′ and annexin II, in aPL-mediated cell activation have been investigated. Moreover, the crucial role of p38 mitogen-activated protein kinase on aPL-mediated cell activation has been demonstrated. Those extensive researches are helping to unveil the etiopathology of APS, ultimately leading to specific treatment for the affected patients.

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Authors and Affiliations

  1. Department of Medicine II, Hokkaido University Graduate School of Medicine, N-15 W-7, Kita-ku, Sapporo, 060-8638, Japan

    Tatsuya Atsumi, Olga Amengual & Takao Koike

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  1. Tatsuya Atsumi
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  2. Olga Amengual
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  3. Takao Koike
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Editors and Affiliations

  1. Department of Pathology, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan

    Kenzo Tanaka M.D., Ph.D. (Professor Emeritus) (Professor Emeritus)

  2. Department of Biochemistry, University of Washington, J317 Health Science Center, Box 357350, Seattle, WA, 98195, USA

    Earl W. Davie Ph.D. (Professor) (Professor)

  3. School of Medicine, Internal Medicine, Keio University, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan

    Yasuo Ikeda M.D., Ph.D. (Professor) (Professor)

  4. Department of Biology, Kyushu University, 6-10-1 Hakozaki, Higashi-ku, Fukuoka, 812-8581, Japan

    Sadaaki Iwanaga Ph.D. (Professor Emeritus) (Professor Emeritus)

  5. Department of Medicine, Nagoya University, 26 Tsurumai-cho, Showa-ku, Nagoya, 466-8550, Japan

    Hidehiko Saito M.D., Ph.D. (Professor Emeritus) (Professor Emeritus)

  6. Pathophysiological and Experimental Division, Department of Pathology, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan

    Katsuo Sueishi M.D., Ph.D. (Professor) (Professor)

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Atsumi, T., Amengual, O., Koike, T. (2008). Etiopathology of the Antiphospholipid Syndrome. In: Tanaka, K., Davie, E.W., Ikeda, Y., Iwanaga, S., Saito, H., Sueishi, K. (eds) Recent Advances in Thrombosis and Hemostasis 2008. Springer, Tokyo. https://doi.org/10.1007/978-4-431-78847-8_35

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