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Pulmonary Hypertension and Antiphospholipid Antibodies

  • Chapter
Hughes Syndrome

Abstract

Pulmonary hypertension (PH) is defined as a mean pulmonary artery pressure greater than 25 mmHg [1, 2]. After many years of debate, it is now agreed that PH can be classified according to three features: anatomical localization of vascular disorder, presence or not of any associated disease, and severity, with the magnitude of reduction of cardiac output as the best predictor survival (1, Table 10.1). The term primary pulmonary hypertension (PPH) has been used extensively in literature, leading to some confusion. PPH usually means that diverse mechanisms have been ruled out, especially chronic causes of hypoxia, left ventricular failure, and repeated pulmonary embolism, and that plexogenic arteriopathy can be found on histological lung examination. PPH is a rare but life-threatening condition, whose pathophysiology has remained mysterious for a while. Recent advances have suggested the importance of diverse factors, such as: imbalance in vasoactive agents — i.e. deficiency of nitric oxide and prostacyclin synthase versus overexpression of endothelin-1 -, vascular endothelial growth factor (VEGF) expression, K+ channel anomalies, genetic susceptibility, and, last but not least, clonal expansion of endothelial cells in primary but not secondary PH [2–6]. Though PPH frequently remains “unexplained”, several comorbid conditions have been identified as possible etiologies, with human immuno-deficiency virus (HIV) infection, prior use of anorectic agents, and connective tissue diseases (CTD) as leaders [2, 7, Table 10.1]. Whatever the “cause”, severe PH may be complicated by (a) superimposed in situ thromboses affecting distal pulmonary arteries [8] and, (b) the development of plexogenic lesions, both thought to occur as a consequence of chronic endothelial injury [1, 6].

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  1. J.-C. Piette
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  2. B. J. Hunt
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Editors and Affiliations

  1. Lupus Research Unit, St Thomas’ Hospital, Lambeth Palace Road, SE1 7EH, London, UK

    M. A. Khamashta MD, MRCP, PhD (Deputy Head) (Deputy Head)

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Piette, JC., Hunt, B.J. (2000). Pulmonary Hypertension and Antiphospholipid Antibodies. In: Khamashta, M.A. (eds) Hughes Syndrome. Springer, London. https://doi.org/10.1007/978-1-4471-3666-8_10

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  • DOI: https://doi.org/10.1007/978-1-4471-3666-8_10

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