Sjögren’s Syndrome in Clinical Practice

84 Pages·2014·2.126 MB·English

by  Graham Hughes, Shirish Sangle, Simon Bowman (auth.)

Graham Hughes
Shirish Sangle
Simon Bowman
Sjögren’s Syndrome
in Clinical Practice
123
Sjögren’s Syndrome
in Clinical Practice

Graham Hughes (cid:129) Shirish Sangle
Simon Bowman
Sjögren’s Syndrome
in Clinical Practice
Graham H ughes Simon Bowman
The London Lupus Centre Department of Rheumatology
London Bridge Hospital Queen Elizabeth Medical
London Centre
United Kingdom Birmingham
United Kingdom
Shirish Sangle
Louise Coote Lupus Unit
St Thomas’ Hospital
London
United Kingdom
ISBN 978-3-319-06058-3 ISBN 978-3-319-06059-0 (eBook)
DOI 10.1007/978-3-319-06059-0
Springer Cham Heidelberg New York Dordrecht London
Library of Congress Control Number: 2014940967
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Contents
1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
What Is Sjögren’s Syndrome? . . . . . . . . . . . . . . . . . . . 1
History. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2
What Are the Symptoms?. . . . . . . . . . . . . . . . . . . . . . . 4
2 Eyes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7
Symptoms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7
Signs. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8
Tests for Eye Involvement . . . . . . . . . . . . . . . . . . . . . . 9
Schirmer’s Test . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9
Slit Lamp Examination . . . . . . . . . . . . . . . . . . . . . . 9
3 Mouth and Salivary Glands . . . . . . . . . . . . . . . . . . . . . 11
4 Lungs. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15
5 Skin . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17
Dryness . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17
Rashes. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17
Raynaud’s Phenomenon. . . . . . . . . . . . . . . . . . . . . . . . 18
Sun Sensitivity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19
Purpura . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22
Livedo . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 23
6 Gut. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27
Swallowing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27
Stomach. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27
Small and Large Bowel. . . . . . . . . . . . . . . . . . . . . . . . . 28
Coeliac Disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28
v
vi Contents
Other ‘Food Allergies’ . . . . . . . . . . . . . . . . . . . . . . 28
Low Thyroid. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29
Hughes Syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . 29
Pancreas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29
7 Bladder, Vagina, Kidney. . . . . . . . . . . . . . . . . . . . . . . 31
Bladder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31
Vagina . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31
Kidney. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32
Therapy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34
8 The Nervous System. . . . . . . . . . . . . . . . . . . . . . . . . . 35
Brain and Spinal Cord . . . . . . . . . . . . . . . . . . . . . . . . 35
Trigeminal Neuralgia. . . . . . . . . . . . . . . . . . . . . . . . . . 36
Other Cranial Nerves . . . . . . . . . . . . . . . . . . . . . . . . . 36
Peripheral Neuropathy. . . . . . . . . . . . . . . . . . . . . . . . 36
Autonomic Neuropathy . . . . . . . . . . . . . . . . . . . . . . . 37
9 Joints, Tendons, Muscles. . . . . . . . . . . . . . . . . . . . . . . 39
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39
Joints . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39
Sjögren’s and Rheumatoid Arthritis. . . . . . . . . . . . . 40
Tendons. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40
Muscles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 41
10 Sjögren’s and Lymphoma. . . . . . . . . . . . . . . . . . . . . . 43
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43
Features of Lymphoma. . . . . . . . . . . . . . . . . . . . . . . . 44
Relationship Between Lymphoma,
Sjögrens, Lupus and Vitamin D. . . . . . . . . . . . . . . . . . . 45
11 Sjögren’s Overlaps. . . . . . . . . . . . . . . . . . . . . . . . . . . . 47
Sjögren’s and Allergy . . . . . . . . . . . . . . . . . . . . . . . . . 47
Sjögren’s and Hughes Syndrome. . . . . . . . . . . . . . . . 47
Sjögrens and Thyroid Disease . . . . . . . . . . . . . . . . . . 48
Sjögrens and Lupus. . . . . . . . . . . . . . . . . . . . . . . . . . . 49
Contents vii
Sjögren’s, Pregnancy, and Newborn
(Anti Ro (SSA) and La (SSB) Antibodies) . . . . . . 49
Sjögrens and Cryoglobulinemia. . . . . . . . . . . . . . . . . 51
Sjögrens and Fibromyalgia. . . . . . . . . . . . . . . . . . . . . 51
12 General Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . 53
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 53
Diet . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54
Eyes. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54
Mouth . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55
Lifestyle . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 56
13 Drug Treatment. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 57
Plaquenil (hydroxychloroquine) . . . . . . . . . . . . . . . . 57
Benefits and Drawbacks of Plaquenil. . . . . . . . . . . . 58
Benefits. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 58
Problems. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59
Mepacrine (Also Called Quinacrine). . . . . . . . . . . . 59
Disease Modifying Agents – DMARDs. . . . . . . . . . 59
Steroids. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59
Azathioprine . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60
Cyclophosphamide . . . . . . . . . . . . . . . . . . . . . . . . . 60
Methotrexate . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 61
Mycophenolate Mofetil . . . . . . . . . . . . . . . . . . . . . 61
Biologics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 61
14 Diagnosis I: Blood Tests. . . . . . . . . . . . . . . . . . . . . . . 63
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 63
Antibodies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 63
Other Blood Tests . . . . . . . . . . . . . . . . . . . . . . . . . . . . 65
ESR. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 65
CRP – ‘C-Reactive protein’. . . . . . . . . . . . . . . . . . . . 65
Full Blood Count. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 65
Anaemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 66
Rheumatoid Factor . . . . . . . . . . . . . . . . . . . . . . . . . . . 66
Cryoglobulins. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 66
viii Contents
15 Diagnosis II: Other Tests . . . . . . . . . . . . . . . . . . . . . . 69
Schirmers Test . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 69
Rose Bengal/Lissamine Green Test . . . . . . . . . . . . . 69
Scintigraphy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 70
Sialography. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 70
Lip Biopsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 70
Ultrasound and MRI Scanning . . . . . . . . . . . . . . . . . 71
16 Causes of Sjögrens . . . . . . . . . . . . . . . . . . . . . . . . . . . 73
Immunology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 73
Hormonal . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 74
Viruses. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 74
Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 75
Apoptosis and Sjögren syndrome . . . . . . . . . . . . . . . 76
Environmental Factors . . . . . . . . . . . . . . . . . . . . . . . . 76
Further Reading Websites . . . . . . . . . . . . . . . . . . . . . . . . . 77
Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 79
Chapter 1
Introduction
What Is Sjögren’s Syndrome?
At its most basic, Sjögren’s Syndrome consists of the com-
bination of dry eyes, dry mouth and aches and pains. It is
now recognised that the underlying abnormality is a mal-
function of the immune system, and that the condition can
affect other organs.
It is also recognised now that Sjögren’s Syndrome is com-
mon and an important cause of aches, pains and fatigue. It is
considered by many doctors to be central to the group of
so-called ‘autoimmune’ diseases, overlapping with other
important disorders such as Hashimoto’s disease (underactive
thyroid). Hughes Syndrome (antiphospholipid syndrome)
and rheumatoid arthritis (Fig. 1 .1 ).
The dryness of the eyes and of the mouth are caused by
inflammation and/or damage to the lachrymal (tear) and sali-
vary glands respectively, the glands being infiltrated by
immune ‘white’ cells (also called ‘lymphocytes’) that go round
the blood stream, lymph nodes, spleen and bone marrow fight-
ing infections such as bacteria or viruses.
A lthough the precise prevalence is not known, it is esti-
mated that about 1 in a 1,000 to 1 in 200 adult women in the
UK have Sjögren’s syndrome. It is, however, rare in men with
approximately 1 man for every 13 women with the condition
(Fig. 1.2 ). A recent epidemiological study by a French group
G. Hughes et al., Sjögren’s Syndrome in Clinical Practice, 1
DOI 10.1007/978-3-319-06059-0_1,
© Springer International Publishing Switzerland 2014