Hughes Syndrome: Highways and Byways

By Graham Hughes and Munther A Khamashta

Hughes Syndrome: Highways and Byways is a handy and easy-to-read guide to the main features of Hughes syndrome. There has been worldwide interest in this 'new' syndrome (first described in 1983). A clotting tendency which can potentially affect any organ in the body, it is:

*         responsible for 1 in 5 recurrent miscarriages (now successfully treated with aspirin and heparin)

*         responsible for 1 in 5 young (under 45) strokes

*         a major cause of early heart attacks

*         responsible for 1 in 5 deep vein thromboses

*         an important cause of migraine.

 

Hughes Syndrome: Highways and Byways is a valuable resource for professionals with an interest in the subject, as well as general practitioners and patients.

• Language: English
• Publisher: Springer
• Release date: Jul 1, 2013
• ISBN: 9781447151616

Graham Hughes

Graham Hughes is a freelance copy-editor and proofreader based in Chester, England. He is also the author of the ebook A Devilish Pastime, a history of all forms of football – originally published in print (as A Develyshe Pastime), by Sportsbooks in 2009. He has additionally contributed to the When Saturday Comes magazine, and to websites such as TimeTravel-Britain.com, Buzzle.com and eSports Media Group.

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Graham Hughes and Munther A. KhamashtaHughes Syndrome: Highways and Byways201310.1007/978-1-4471-5161-6© Springer-Verlag London 2013

Graham Hughes and Munther A. Khamashta

Hughes Syndrome: Highways and Byways

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Graham Hughes

The London Lupus Centre, London Bridge Hospital, London, UK

Munther A. Khamashta

Division of Women’s Health, Graham Hughes Lupus Research Laboratory, King’s College London, London, UK

ISBN 978-1-4471-5160-9e-ISBN 978-1-4471-5161-6

Springer London Heidelberg New York Dordrecht

Library of Congress Control Number: 2013943331

© Springer-Verlag London 2013

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Preface

In the 30 years since the description of the antiphospholipid syndrome (APS/Hughes syndrome), many clinical features have been added, emphasising the truly multisystem nature of the condition.

Hughes syndrome is a challenge for clinicians for one reason in particular – its predilection to cross specialty borders. And because laboratory investigation of the syndrome is still far from perfect, the need for painstaking clinical observation is critical.

In this book, we have attempted to highlight the many aspects of Hughes syndrome, some, like miscarriage and migraine, from the ‘highways’ and some more ‘small print’ features. Where possible, we have included a review reference or two for the reader to follow up in more detail.

Graham Hughes

Munther A. Khamashta

Contents

1 The Nervous System 1

1.1 Migraine 1

1.2 Memory Loss 2

1.3 Stroke 3

1.4 Seizures 4

1.5 Temporal Lobe Epilepsy 5

1.6 Myelopathy 5

1.7 Multiple Sclerosis 6

1.8 Tics and Movements 7

1.9 Devic’s Disease 8

1.10 Autonomic Neuropathy 9

1.11 Intracranial Hypertension 9

2 Neuropsychiatric 11

2.1 Sleep Disturbance 11

2.2 Alzheimer’s 11

2.3 Psychosis, Depression, Autism and Obsessive Compulsive Disorder 13

2.4 Visual Loss 14

3 Heart 15

3.1 Angina and Syndrome X 15

3.2 Myocardial Infarction 16

3.3 Arrhythmias 16

3.4 Heart Valve Lesions 18

3.5 Intracardiac Thrombosis 20

3.6 Myocarditis 20

4 Arteries and Veins 21

4.1 Arterial Thrombosis 21

4.2 Focal Arterial Narrowing 22

4.3 Accelerated Atheroma 22

4.4 Veins 23

5 Kidney 25

5.1 Renal Infarction 25

5.2 Renal Artery Stenosis 25

5.3 Microvascular Thrombosis 27

5.4 Renal Vein Thrombosis 29

6 Liver and Gastrointestinal Tract 31

6.1 Liver Vein Thrombosis 31

6.2 Budd–Chiari Syndrome 31

6.3 Abnormal Liver Tests 33

6.4 Cirrhosis 33

6.5 Coeliac Axis Stenosis 34

6.6 Bowel Ischaemia 36

7 Ear, Nose and Throat 37

7.1 Balance Problems 37

7.2 Tinnitus 38

8 Lungs 39

8.1 Pulmonary Embolism 39

8.2 Pulmonary Hypertension 40

9 Musculoskeletal System 41

9.1 Avascular Necrosis 41

9.2 Arthralgia and Myalgia 42

9.3 Fracture 43

9.4 Spinal Stenosis 44

9.5 Reflex Sympathetic Dystrophy 44

10 Skin 47

10.1 Livedo Reticularis 47

10.2 Skin Ulcers 48

10.3 Cutaneous Gangrene 48

10.4 Splinter Haemorrhages 49

11 Pregnancy 51

11.1 Miscarriage 51

11.2 Fetal Death 51

11.3 Intrauterine Growth Restriction and Pre-eclampsia 52

11.4 Infertility 53

12 Blood 55

12.1 Low Platelets 55

12.2 Anaemia 56

12.3 Disseminated Intravascular Coagulation (DIC) 56

13 Tests for Hughes Syndrome 59

13.1 Anticardiolipin Antibodies 59

13.2 Lupus Anticoagulant 60

13.3 ß2-Glycoprotein 1 (ß2GP1) 61

13.4 Other Antibodies 62

14 Associated Conditions 63

14.1 Lupus 63

14.2 Sjogren’s Syndrome 64

14.3 Thyroid 65

14.4 Crohn’s Disease 66

14.5 Other Associated Conditions 67

14.6 Catastrophic APS 68

15 Aetiology 71

15.1 Autoimmunity 71

15.2 Genetics 71

15.3 Environmental 72

15.3.1 The Pill 72

15.3.2 Diet 72

15.4 Hughes Syndrome and Flying 73

15.5 Infection 74

16 Diagnosis and Classification 75

16.1 Diagnosis 75

16.2 Classification 76

16.3 ‘Seronegative APS’ 77

17 Treatment 79

17.1 Aspirin 79

17.2 Heparin 80

17.3 Warfarin (Coumadin) 81

17.4 Newer Anticoagulants 82

17.5 Intravenous Immunoglobulin 83

17.6 Immunosuppressants 84

17.7 Biologics 84

17.8 Plasma Exchange 85

17.9 Plaquenil 86

18 The Future 89

Index91

Graham Hughes and Munther A KhamashtaHughes Syndrome: Highways and Byways201310.1007/978-1-4471-5161-6_1© Springer-Verlag London 2013

1. The Nervous System

Graham Hughes¹  and Munther A. Khamashta²

(1)

The London Lupus Centre, London Bridge Hospital, London, UK

(2)

Division of Women’s Health, Graham Hughes Lupus Research Laboratory, King’s College London, London, UK

Abstract

Second only to obstetrics and the risk of pregnancy, the nervous system appears to be the major organ involved in