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Sjogren's syndrome

Introduction

Mayo Clinic description

International classification criteria for Sjögrens syndrome include ocular symptoms, oral symptoms, ocular signs, histopathology, salivary gland involvement and sialography. The classification requires four of the six items, one of which must be positive minor salivary gland biopsy or a positive antibody test. Early diagnosis is important to prevent further complications. 1)

Primary Sjögren's syndrome (pSS) is a chronic systemic autoimmune disease, of slow progression, characterized by lymphocytic infiltration of the exocrine glands, that leads to sicca symptoms, mainly xerophtalmia and xerostomia. It may involve any organ and lead to extraglandular manifestations, which also can precede typical glandular manifestations and delay the diagnosis of pSS. In the past years, better knowledge of the disease has led to improvement in treatment management. 2)

Evidence of infectious cause

Identification of Hepatitis D Virus in primary Sjögren's syndrome patients and induction of a complete pSS-like phenotype in vivoA type of scientific study that analyzes an organism in its natural living environment. provides further support of a viral-mediated etiopathology in the development of pSS. 3)

Recent Research

According to literature, Sjogren's Syndrome (SS) and Systemic Lupus Erythematosus (SLE) are the most frequently reported diseases associated with Neuromyelitis Optica Spectrum Disorders among systemic autoimmune diseases. 4)

These results showed that IL-6 stimulation induced REG Iα transcription through STAT3 activation and binding to the consensus sequence of REG Iα promoter in salivary ductal cells. This IL-6/STAT dependent REG Iα induction might play a role in the pathogenesis of SS.5)

Mercury exposure was associated with increased titers of several autoantibodies in serum including anti-GSTA1. These proteins play a wide variety of roles, including as antioxidants, in the regulation of pro- and anti-inflammatory cytokinesAny of various protein molecules secreted by cells of the immune system that serve to regulate the immune system., as well as danger and oxidative stress signaling. Dysregulation of these proteins and pathways is believed to play a role in autoimmune diseases such as rheumatoid arthritis, Sjögren׳s syndrome, and multiple sclerosis. 6)

Although over one hundred autoimmune diseases have been investigated in relation to H. pylori, we discuss a select number of papers with a larger literature base, and include Sjögrens syndrome, rheumatoid arthritis, systemic lupus erythematosus, vasculitides, autoimmuneA condition or disease thought to arise from an overactive immune response of the body against substances and tissues normally present in the body skin conditions, idiopathic thrombocytopenic purpura, autoimmune thyroid disease, multiple sclerosis, neuromyelitis optica and autoimmune liver diseases. 7)

pSS patients have impaired blood pressure response to standing. Dysautonomia correlates with PSS-associated symptoms and quality of life. 8)

Neurologic disease is a common extraglandular manifestation of Sjögren's syndrome 9)

This information aids understanding of the history and development of dry eye research, in addition to the impact and characteristics of the contributors to the field. 10)

The purpose of this study was to investigate salivary tissue assessment with various sonoelastographic modalities (real-time tissue elastography, Virtual Touch imaging and quantification) in patients with Sjögren's syndrome as compared with an appropriate control group. 11)

Abnormalities of memory B cells seem to be closely involved in the pathogenesis of primary Sjögrens Syndrome (pSS) and its malignant complication, B cell lymphoma. Recent studies on B cells in pSS add to our understanding of the distinct memory B cell subsets in pSS. Reduction of peripheral memory CD27(+) B cells, most strikingly of the CD27(+)IgM(+) subset, may indicate a lack of appropriate censoring mechanisms and incomplete differentiation processes within the ectopic lymphoid tissues in pSS. This ectopically formed lymphoid tissue might protect autoreactive memory B cells from deletion by physiological check-points and, thereby, may contribute to the perpetuation of the disease as well as to an enhanced lymphoma risk. 12)

The presence of Sjögren's syndrome influences the expression of the other autoimmune disease to some degree, for instance by increasing fatigue and lymphoma risk. 13)

This report presents a patient with Devics neuromyelitis optica associated with primary Sjögrens syndrome. Her first attack was right-sided optic neuritis at age 10 years. Attacks involving both optic nerves and medulla spinalis were recorded during the ensuing years. The diagnosis of Sjögrens syndrome could not be made until the second decade because it was not suspected. 14)

The quality of life of patients with Sjogrens syndrome often is degraded further by serious, multisystemic manifestations, and they are subject to a forty-fold increased risk of developing B cell lymphomas. In normal lacrimal glands, secretory epithelial cells, autoimmune effector lymphocytes, and regulatory lymphocytes can be seen as collaborating to maintain a local immunohomeostasis. 15)

OBJECTIVES: To develop a questionnaire for the assessment of health-related quality of life (HRQL) in primary Sjögren's syndrome (PSS), and to test its psychometric properties.16)

Patient interviews

Bonnie B.

lupus, Sjogren’s Syndrome

Read the interview


Interviews of patients with other diseases are also available.

I was diagnosed with Sjogrens at the time of inquiring with my doctor about MP. My eyes were red, my saliva was increasingly scanty such that I could only get ½ way through a serving of potato, my favorite food, if I was very lucky.

It must have been the latest of my 'auto-immune' diseases to kick in, as it began to clear up early. After a year on OLM 40mg six hourly, my eyes were no longer red.. I can now eat potato although I still need to take it slowly. - Sallie Q

see The eyes have it (Registration required)

Patient experiences and discussion from Study Site topic(s)

“I started to suffer from my eyelids sticking to my eyes in the night two years into the MP (I am now a few months into year 5). At first, it was just that and brought on by a sleep med in the same family as Valium, which I had to stop using after a couple of months because it became ineffective. I now find that any drug like Valium makes the situation worse. I also found that if I gently rubbed my eyelids (if I remembered to do that)before opening, then I would be fine (or relatively fine). I used coconut oil before bed when it was an every night problem and that took care of it.

eC posted: Mon Mar 21st, 2011to E-N

Now, however, the coconut oil seems to aggravate. Also, now, I appear to be producing scratchy crystals under my eyelids at night and so gently rubbing my eyelids are a huge NO NO. Instead, I find that I have to use artificial tears (I use the kind that have individual portions b/c I find them easier to negotiate when I can't open my eyes to see) before opening my eyes. I just apply the artificial tears to the corner of my eyes and allow them to seep in. I don't have to do this every morning… sometimes just lying there and waiting will allow me to open my eyes. On some mornings, though, they are painfully stuck and not would do but use the artificial tears.”

“My eyes have been a problem for a long time. First I felt that I had fibers in my eyes and was constantly trying to find them but never could. For years I blamed it on my woolen blanket. But, then when the fibers started to turn into glasslike shards I realized it was part of my Lyme symptoms. For a while, before I was on the MP, I needed to use eye drops many times during the day…my eyes were severely dry and irritated, but always the worst in the am. My doctor didn't believe me when I told him I had glass-like shards…he thought I was joking. Crystals is a better word, but they feel like glass to me. My eyes are constantly changing as I proceed through the MP. I can go for long periods with no problems at all, and then, the grittiness and the stickiness return, usually accompanied by blurred vision and other herx symptoms.

The only thing that seems to help is the natural tears eye drops.

I put the sandlike grains that my skin used to exude and the crystals in my eyes into the same category. The sand has stopped…so I am hoping the gritty eyes will eventually as well.” A D Mar 23rd, 2011

“Dry Mouth / Sjogrens - For years I had tenderness in my salvary glands under the ears, I never understood why this area was so tender to the touch. I also randomly started getting numerous cavities in my late 20's with no explanation as I'd never had a cavity in my life, my diet was better than in my younger years and my dental habits hadn't changed. While on the protocol I tested positive for the SSB antibodies on an ENA test and it all of a sudden made sense. My salivary glands were being attacked by my immune system. On the marshall protocolA curative medical treatment for chronic inflammatory disease. Based on the Marshall Pathogenesis. this tenderness gradually went away. Once I stopped the protocol, I noticed that I had a dry mouth and was choking on things frequently, to the point that I had to throw up at times. My gums and teeth were also becoming tender due to the lack of saliva to clean my mouth. My salivary glands were obviously not secreting properly and they were becoming tender again. Back on the protocol and this all became a non issue.” Jair posted Dec. 2014

“ for months and months maybe years before I started MP, I experienced gritty eyes. I always thought I had something in the eye as it would come and go, also I live in a windy dirty part of a big city. I had never heard of Sjogrens syndrome, and when I went to my MP doctor to complain of mental problems including memory loss and severe irritability (temper tantrum) my hubbie asked for a diagnosis and we were told Sjogrens ….it has been a real eye opener.” SQ posted: Tue May 3rd, 2011

eC posted “I've discovered that eating lettuce consistently makes the eyelid sticking to the eyeball problem much worse. I can have lettuce occasionally, just not consistently. Yet another thing to remove from my diet to keep my IP tolerable.”

TC Posted: Sat Sep 1st, 2012 “I found information that said many bacteria, microbes etc accumulate on the eyelashes. I think that is what our eyelashes are meant to do to protect our eyes and keep that stuff out of them.

When my eyes get itchy and watery, I gently scrub my eyelashes with a gentle soap and water 3-4 times a day or as often as needed. It works for me.”

SQ posted “Lee recommended washing eyelashes in baby shampoo I was about to go and look for “No More Tears”, the only name I knew of when I noticed my daughter had left 'Aveeno, Ultra calming Foaming Cleanser' next the basin” :-)

“I've actually gotten over many/most of my Sarc/Sjogren's symptoms that I formerly suffered from - and severe photosensitivityAbnormal sensitivity to sunlight and bright lights. Also referred to as "sun flare" or "light flare." - gone, severe joint pain from light exposure - gone, fatigue, hardly at all - on days where I've slept enough.” SanDiegoJoy posted Nov.2012 (during 8th year, her Sjogren's Sx were worse from 5th to 7th year)

Traditional Chinese medicines used in primary Sjogren's syndrome patients

The aOR for those suffering from at least one rheumatologic disease was 1.56 (95% CI: 1.26-1.93), those with two rheumatologic diseases was 1.98 (95% CI: 1.29-3.04), while those with three or more rheumatologic diseases was 7.86 (95% CI: 1.09-56.58). 17)

Notes and comments

<DiseaseHierarchy>

GETCONTENT San Diego Joy

1)
Sjögren's Syndrome: A Case Study.
Jadhav S, Jadhav A, Thopte S, Marathe S, Vhathakar P, Chivte P, Jamkhande A
J Int Oral Health7p72-4(2015 Mar)
2)
[Management of the extraglandular manifestations of primary Sjögrens syndrome].
Rosas Gómez de Salazar J, Senabre Gallego JM, Santos Ramírez C
Reumatol Clin6 Suppl 2p6-11(2010 Sep)
3)
Hepatitis Delta Virus Detected in Salivary Glands of Sjögren's Syndrome Patients and Recapitulates a Sjögren's Syndrome-Like Phenotype <i>in Vivo</i>.
Weller ML, Gardener MR, Bogus ZC, Smith MA, Astorri E, Michael DG, Michael DA, Zheng C, Burbelo PD, Lai Z, Wilson PA, Swaim W, Handelman B, Afione SA, Bombardieri M, Chiorini JA
Pathog Immun1p12-40(2016 May)
4)
Autoimmune diseases associated with Neuromyelitis Optica Spectrum Disorders: A literature review.
Shahmohammadi S, Doosti R, Shahmohammadi A, Mohammadianinejad SE, Sahraian MA, Azimi AR, Harirchian MH, Asgari N, Naser Moghadasi A
Mult Scler Relat Disord27p350-363(2018 Nov 16)
6)
Novel biomarkers of mercury-induced autoimmune dysfunction: a cross-sectional study in Amazonian Brazil.
Motts JA, Shirley DL, Silbergeld EK, Nyland JF
Environ Res132p12-8(2014 Jul)
7)
Helicobacter pylori and autoimmune disease: cause or bystander.
Smyk DS, Koutsoumpas AL, Mytilinaiou MG, Rigopoulou EI, Sakkas LI, Bogdanos DP
World J Gastroenterol20p613-29(2014 Jan 21)
8)
Primary Sjogrens syndrome is associated with impaired autonomic response to orthostasis and sympathetic failure.
Ng WF, Stangroom AJ, Davidson A, Wilton K, Mitchell S, Newton JL
QJM105p1191-9(2012 Dec)
9)
Neurological Complications of Sjögren's Syndrome: Diagnosis and Management.
McCoy SS, Baer AN
Curr Treatm Opt Rheumatol3p275-288(2017 Dec)
10)
Citation analysis of the dry eye literature.
Nichols JJ
Ocul Surf11p35-46(2013 Jan)
11)
Sonoelastographic Modalities in the Evaluation of Salivary Gland Characteristics in Sjögren's Syndrome.
Hofauer B, Mansour N, Heiser C, Gahleitner C, Thuermel K, Bas M, Knopf A
Ultrasound Med Biol42p2130-9(2016 Sep)
12)
What do we know about memory B cells in primary Sjögren's syndrome?
Hansen A, Daridon C, Dörner T
Autoimmun Rev9p600-3(2010 Jul)
13)
Relationship of Sjögren's syndrome to other connective tissue and autoimmune disorders.
Theander E, Jacobsson LT
Rheum Dis Clin North Am34p935-47, viii-ix(2008 Nov)
14)
Primary Sjögrens syndrome presenting as neuromyelitis optica.
Gökçay F, Celebisoy N, Gökçay A, Kabasakal Y, Oder G
Pediatr Neurol36p58-60(2007 Jan)
16)
Assessing health-related quality of life in primary Sjögren's syndrome-The PSS-QoL.
Lackner A, Stradner MH, Hermann J, Unger J, Stamm T, Graninger WB, Dejaco C
Semin Arthritis Rheum48p105-110(2018 Aug)
17)
The traditional Chinese medicine prescription patterns of Sjögren׳s patients in Taiwan: a population-based study.
Yu MC, Lin SK, Lai JN, Wei JC, Cheng CY
J Ethnopharmacol155p435-42(2014 Aug 8)
home/diseases/sjogrens.txt · Last modified: 01.11.2019 by sallieq
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