| Sjögren's
Syndrome |
| This page
was written by Shelley
Ensz, and has not yet been medically edited. Scleroderma
(SD) affects everyone differently. See Disclaimer. |
|
|
| What is Sjögren's
Syndrome? |
 Hallmark symptoms
of Sjögren's include dry
eyes, dry
mouth, dry
vagina. Sjögren's can also cause fatigue,
and it can affect the skin, lungs, heart, kidneys, muscles,
and central nervous system. People can also have any of
the above symptoms separately, or due to other
conditions.
Ninety percent of Sjögren's patients are female. Many
Sjögren's patients are undiagnosed, with an average
of ten years from onset to diagnosis—for those fortunate
enough to be diagnosed at all. |
| Typical diagnostic
tests for Sjogren's include Schirmer's eye test, slit-lamp
test, tests measuring saliva flow, radiologic salivary scans,
and salivary gland biopsy. |
| "Sjögren's may also be
referred to as: Dacryosialoadenopathia atrophicans, Gougerot-Houwer-Sjögren,
Gougerot-Sjögren, Keratoconjunctivitis Sicca, Keratoconjunctivitis
sicca-xerostomia, Secreto-inhibitor-xerodermostenosis, and
Sicca Syndrome." NORD |
| Sjögren's
Syndrome is a "chronic, systemic inflammatory disorder
of unknown cause, characterized by dryness of the mouth,
eyes, and other mucous membranes and often associated with
rheumatic disorders sharing certain autoimmune features (eg,
RA, scleroderma, SLE) and in which lymphocytes infiltrate
mucosal and other tissues." Merck Manual |
| Sjögren's
Syndrome About.com |
| Sjögren’s
Syndrome. Sjögren's (SHOW-grins) syndrome is an autoimmune
disease that attacks the exocrine glands (i.e., moisture-producing
glands) of the body, specifically the lacrimal and salivary
glands. U.S. Pharmacist. Vol. No: 32:3 Posted: 3/21/2007. |
| Sjögren's
syndrome (SS) is associated with and not secondary to systemic
sclerosis (SSc). SS associated with SSc has the same
features as pSS, but SSc seems to be less serious. Moreover,
the association of SS and SSc is frequently accompanied by
a spreading of autoimmunity. Rheumatology. Vol 46, Number
2. Pp. 321-326. Feb 07. |
| Sjogren's
syndrome (SS) in dermatology. SS is a systemic autoimmune
disease characterized by dry eyes and dry mouth. The dermatologic
manifestations range from drynessness (sicca) and its complications
to vasculitis. PubMed. Clin Dermatol. 2006 Sep-Oct;24(5):393-413. |
| Sjögren's
Syndrome eMedicine Journal |
| Symptoms of Sjogren's
Syndrome |
| Hallmark symptoms of Sjögren's
include dry
eyes, dry
mouth, dry
vagina. Sjögren's can also cause fatigue,
and it can affect the skin, lungs, heart, kidneys, muscles,
and central nervous system. People can also have any of the
above symptoms separately, or due to other
conditions. |
|
|
| Diagnosis of Sjogren's
Syndrome |
| Diagnosis of Sjogren's Syndrome. Typical diagnostic tests for Sjogren's
include Schirmer's eye test, slit-lamp test, tests measuring
saliva flow, radiologic salivary scans, and salivary gland
biopsy. ISN. |
|
|
| Similar and Overlapping
Conditions |
| Sjogren's symptoms have been associated
with other conditions, including autoimmune or collagen vascular
diseases, such as thyroid
disease, gastic
antral vascular ectasia (watermelon stomach), systemic
scleroderma, lupus, Reynold's
syndrome, primary
biliary cirrhosis, and rheumatoid
arthritis. |
| About twenty percent of patients
with systemic
scleroderma also have Secondary Sjögren's Syndrome. |
| Sjogren's
syndrome is associated with and not secondary to systemic
sclerosis. SS associated with SSc has the same features
as pSS (primary Sjogren's Syndrome), but SSc seems to be
less serious. The association of SS and SSc is frequently
accompanied by a spreading of autoimmunity. PubMed. Rheumatology
(Oxford). 2006 Jul 28. |
| Prevalence
of Sicca Symptoms and Sjogren's Syndrome in Systemic Sclerosis:
Results of a Prospective Two-Center Series of 133 Patients. The
prevalence of sicca symptoms and SS, using international
classification criteria for SS, has been found respectively
in 68% and 14% of SSc patients.J. Avouac. FRI0318 EULAR
2006. (Also see: Limited
Scleroderma) |
 Scleroderma
Dental Involvement Scleroderma can cause many dental
problems, including small mouth, dry mouth, bone reaborption,
receding ligaments, and TMJ. ISN |
| Systemic
sclerosis-associated Sjogren's syndrome and relationship
to the limited cutaneous subtype: Results of a prospective
study of sicca syndrome in 133 consecutive patients. There
was a 68% prevalence of sicca syndrome in this prospective
series of SSc patients. PubMed. Arthritis Rheum. 2006
Jun 26;54(7):2243-2249. (Also see: Limited
Scleroderma) |
| Association
of systemic and thyroid autoimmune diseases. Systemic
and thyroid autoimmune diseases often overlap with each other.
HT (Hashimoto's thyroiditis) and GD (Graves' disease) may
be most common among MCTD, SSc and SS patients. Therefore
it is clinically important to screen patients with systemic
autoimmune diseases for the co-existence of thyroid disorders. PubMed.
Clin Rheumatol. 2005 Oct 25. (Also see: Thyroid
Disease, MCTD,
and What is Scleroderma?) |
| Dry
eyes can cause misery but no tears. Autoimmune diseases
such as lupus, rheumatoid arthritis or Sjogren's syndrome
are the leading causes of the aqueous form, said David Sullivan,
a senior scientist at Schepens. The Boston Globe 2/24/2004.
(Also see: Lupus and Rheumatoid
Arthritis) |
| AUTOIMMUNE:
Identification of masqueraders of autoimmune disease in the
office. There are several rheumatologic and autoimmune
disorders that can masquerade as allergic disease. These
conditions include rheumatoid and juvenile arthritis, Sjogren's
syndrome, systemic lupus erythematosus, Behcet's and antiphospholipid
syndromes, systemic sclerosis, vasculitis, sarcoidosis, chronic
fatigue syndrome, and fibromyalgia. PubMed. Allergy Asthma
Proc. 2003 Nov-Dec;24(6):421-9. (Also see: Rheumatoid
Arthritis, Lupus, Behcet's, Antiphospholipid, Systemic
Sclerosis (Scleroderma), Vasculitis, Sarcoiditis, Fibromyalgia
and CFS) |
| COLLAGEN
DISEASES: Significance of the cardiovascular function tests
in patients with collagen diseases, especially for SSc (systemic
scleroderma). The purpose of this study is to evaluate
the cardiovascular function in patients with incomplete type
of SSc (SSSD; Scleroderma Sjogren syndrome associated Spectrum
Disorders), SSc (Systemic Scleroderma) and SLE (Systemic
Lupus Erythematosus). Iischemic myocardial damage, left ventricular
hypertrophy or QT interval prolongation was observed in 33%
cases of scleroderma associated and 32% cases of SSc, respectively.
Parasympathetic disorders were observed in approximately
20% cases of SSSD, SSc and SLE. PubMed. Rinsho Byori.
2004 May;52(5):401-5. (Also see: Cardiac
Involvement) |
| GAVE:
Gastric Antral Vascular Ectasia (Watermelon Stomach) in a
Patient with Sjögren's Syndrome. Gastric antral
vascular ectasia (GAVE), a rare yet treatable cause of upper
gastrointestinal bleeding, has been described in a variety
of autoimmune diseases. After several fulguration therapies
with argon-plasma coagulator, the mucosal lesions improved
and her hemoglobin levels returned to normal. J Rheumatol
NO. 5 MAY 2003;30:1090-2. (Also see: Watermelon
Stomach) |
| REYNOLD's
SYNDROME: An Association to Remember. This syndrome constitutes
the coexistence of two immunogenetically determined disorders:
systemic scleroderma (CREST syndrome) and primary biliary
cirrhosis (PBC), and it seems to be a favourable factor for
liver disease progression. Association to Sjögren syndrome
(SS) was reported. R. M. Morlà. AB0105 EULAR 2004.
(Also see: CREST and Liver
Involvement) |
| Triad
of thymoma, myasthenia gravis and pure red cell aplasia combined
with Sjogren's syndrome. The case is the first to be
reported with Sjogren's syndrome and the triad of thymoma,
myasthenia gravis and pure red cell aplasia. PubMed. Jpn
J Thorac Cardiovasc Surg. 2004 Jul;52(7):345-8. (Also see: Myasthenia
Gravis) |
| THYROID:
Presence of systemic autoimmune disorders in patients with
autoimmune thyroid diseases (ATD). ANA (antinuclear antibodies)
are detected in 1/3 of patients with autoimmune thyroid disease
(ATD). Anti-dsDNA, anti-Ro, and aCL can also be found in
ANA positive patients with ATD. SS (Sjogren's Syndrome) occurs
in about 1/10 of ANA positive patients with ATD. PubMed.
Ann Rheum Dis. 2004 Sep;63(9):1159-61. (Also see: Thyroid
Disease and Antibodies) |
| THYROID:
Evaluation of salivary gland function in women with autoimmune
thyroid diseases. AITD (autoimmune thyroid diseases )
may be associated with disturbances in salivary secretion
which depends on thyroid hormones production. It can be suggested
that autoimmunological processes within salivary glands may
influence their function. PubMed. Wiad Lek. 2003;56(9-10):412-8.
(Also see: Thyroid
Disease) |
| Treatments for Sjögren's |
|
| Overview of Treatments
for Sjogren's |
| Treatments for Sjogren's may consist
of therapies aimed at relieving specific symptoms, such as
dry eyes or dry mouth, or targeted at trying to control the
underlying disease process. |
| Clinical
Trials: Sjögren's Syndrome CenterWatch |
| Dry.Org
- Internet Resources for Sjögren's Syndrome Dry.Org |
| Renal Involvement and Followup of 130 Patients with Primary Sjögren's Syndrome (pSS). The combination of corticosteroids and immunosuppressors significantly improves the renal function of patients with pSS. J Rheumatol 2008;35:278-84. |
Lack of efficacy of etanercept in Sjögren’s syndrome correlates with failed suppression of TNF{alpha} and systemic immune activation. Etanercept is an ineffective therapeutic agent in pSS consistent with the absence of suppression of TNF and other indicators of immune activation. Ann Rheum Dis. 15 January 2008. |
| A
case of systemic sclerosis and sjogren's syndrome with cardiac
tamponade due to steroid-responsive pericarditis. Pericarditis
due to SSc has been reported unresponsive to steroid therapy,
but several cases of steroid responsive pericarditis due
to SSc or SjS have been reported. PubMed. Arerugi. 2006
Jul;55(7):827-31. (Also see: Cardiac
Involvement) |
| Treatments for Dry Eyes |
| Dry Eyes: Artificial Tears, Dry
Eyes, Tears Naturale, Refresh, Refresh Plus, HypoTears, Lacrisert
artificial tear inserts. |
| Lacrisert. Prescription-only, once-daily, preservative-free Lacrisert® lubricates and protects eyes all day, providing continuous relief from moderate to severe dry eye symptoms all day. In some patients, it may also help slow, stop or reverse further damage to your corneal surface. Lacrisert. |
| Gen-Teal Gel by CIBA Vision is
an over-the-counter gel for dry eyes. It is best used at night,
or along with liquid drops, as it can cause blurriness. |
| Management of Ocular Surface Inflammation in Sjogren Syndrome. Anti-inflammatory therapy in primary Sjogren syndrome significantly improved subjective symptoms and objective ocular signs; however, we did not find evidence that anti-inflammatory treatment increases tear production in patients with Sjogren syndrome. Cornea. 26 Supplement 1:S13-S15, October 2007. |
| Current
treatments of xerophthalmia in Sjogren's syndrome. The
development of topical cyclosporine and other immunomodulating
agents is the most relevant progress in the treatment of
keratoconjunctivitis sicca in Sjogren's syndrome. PubMed.
Rev Med Interne. 2004 May;25(5):376-382. |
| Therapeutic
effect of cevimeline on dry eye in patients with Sjogren's
syndrome: a randomized, double-blind clinical study. These
results indicate that cevimeline, 20 mg three times daily,
is safe and effective in improving symptoms of dry eye in
patients with SS. Additional studies, with larger patient
populations, are needed to further assess the effectiveness
of cevimeline for dry eye. PubMed. Am J Ophthalmol. 2004
Jul;138(1):6-17. |
| Treatments for Dry Mouth |
| Dry Mouth: Biotene products such
as toothpaste, mouthwash, and gel; Salivert, Optimoist, Saliva
Substitute, Moi-Stir, and Salix Lozenges. |
| Dental
Involvement ISN |
| Evoxac was
approved in the U.S. in January 2000 for treatment of dry mouth
in patients with Sjögren's. CenterWatch |
| Green tea may help prevent autoimmune diseases. Green tea may help protect against autoimmune disease. Researchers studied an animal model for type I diabetes and primary Sjogren's Syndrome. Further study could help determine green tea's protective role in other autoimmune diseases, including lupus, psoriasis, scleroderma and rheumatoid arthritis. EurekAlert! 04/19/07. (Also see: Diabetes and Alternative Therapies) |
| Gene
Therapy Rebuilds Bone, Tissue in Dental Patients. Clinical
human gene transfer studies for head and neck cancer treatment
are already underway. Experts in the dental application of
gene therapy say patients with these conditions will be among
the first to undergo gene therapy to repair damaged salivary
glands due to cancer radiation treatment or because of the
autoimmune disease, Sjogren's syndrome. Expired article from U.S. Newswire.
10/21/03. |
| Sjogren's Research |
|
| Overview of Sjogren's Research |
| Sjogren's
syndrome. Hypofunction rather than destruction of these
cells is now regarded as the main mechanism of secretory
failure in Sjogren's syndrome. PubMed. Best Pract Res
Clin Rheumatol. 2004 Jun;18(3):313-29. |
| Gene-altered
mouse is model for rare autoimmune syndrome. By knocking
out a single gene in mice, immunologists at Duke University
Medical Center have mimicked a little-understood autoimmune
disorder in humans. According to researchers, the achievement
not only offers insight into Sjögren's syndrome, but into
the general developmental machinery of the immune system. EurekAlert!
10-19-04. |
| Sjogren's Research: Antibodies |
| Sjögren's Antibodies (Anti-SS-A/Anti-SS-B). SS-A(Ro) is found in 60% to 70% of patients with Sjögren's syndrome and 30% to 40% of patients with SLE. SS-B(La) is found in 50% to 60% of Sjögren's syndrome and 10% to 15% of SLE. LabCorp. |
| Epithelial
CXCR3-B Regulates Chemokines Bioavailability in Normal, but
Not in Sjogren's Syndrome, Salivary Glands. Sjogren's
syndrome (SS) is an autoimmune disease characterized by a
mononuclear cell infiltrate of activated T cells around the
duct in the salivary gland. Epithelial CXCR3 may play a critical
role in the pathogenesis of SS and its agonists may be potential
therapeutic targets. PubMed, J Immunol. 2006 Feb 15;176(4):2581-9. |
| Yale
scientists identify structure for RNA quality control. A
report by Yale scientists in the journal Cell sheds new light
on how the protein Ro, a major autoantigen in patients with
autoimmune disease, recognizes misfolded RNAs, creating a
RNA quality control system for cells. Medical News Today.
06/02/05. (Also see: Lupus
Antibodies ) |
| Sjogren's
syndrome. The salivary and lacrimal glands from patients
with SS harbor unique and highly selected T- and B-cell populations.
B-cell activation may also provide a basis for lymphoma development
observed in up to 5% of the patients with SS. PubMed.
Eur J Oral Sci. 2005 Apr;113(2):101-13. |
| Sjogren's Research: Brain
Involvement |
| An
autopsied case of Sjogren's syndrome with massive necrotic
and demyelinating lesions of the cerebellar white matter. The
combination of Sjogren's syndrome, granulomatous angitis,
and foci of perivenous demyelination suggests that an autoimmune
mechanism played an important role in causing the necrotic
lesions in the cerebellar white matter in this case. PubMed.
J Neurol Sci. 2004 Oct 15;225(1-2):143-8. (Also see: Scleroderma
Brain Involvement) |
| Sjogren's Research: Causes |
| Can low dose diagnostic dental radiation trigger Sjögren’s syndrome? We hypothesize that diagnostic dental radiation could be a factor for the development of SS as a result of either radiation induced apoptosis or through oxidative modification of proteins. Science Direct. Medical Hypotheses Vol 69, Issue 5, 2007, Pages 995-1000. |
| Specific
immunotherapy-induced Sjogren's syndrome. This is the
first case to be reported of Sjogren's syndrome following
SIT (Allergen-specific immunotherapy). Patients undergoing
SIT must be carefully followed up for the development of
autoimmunity and an autoimmune disease. PubMed. Rheumatol
Int. 2005 Jun 17. |
| Hepatitis
C virus, Sjogren's syndrome and B-cell lymphoma: linking
infection, autoimmunity and cancer. Viruses have been
proposed as possible etiologic or triggering agents of systemic
autoimmune diseases, with hepatitis C being one most frequently
associated with autoimmune features and with systemic autoimmunes
such as mixed cryoglobulinemia or Sjogren's Syndrome. Association
between HCV infection and hematologic malignancies, mainly
non-Hodgkin's lymphoma, is supported by several studies. PubMed.
Autoimmun Rev. 2005 Jan;4(1):8-15. (Also see: Liver
Involvement, Cancer
and Scleroderma, and Causes
of Scleroderma: Infection) |
| Sjogren's Research: Dry
Eyes |
| The
role of the lacrimal functional unit in the pathophysiology
of dry eye. Tear secretion is controlled by the lacrimal
functional unit consisting of the ocular surface (cornea,
conjunctiva, accessory lacrimal glands, and meibomian glands),
the main lacrimal gland and the interconnecting innervation.
If any portion of this functional unit is compromised, lacrimal
gland support to the ocular surface is impeded. PubMed.
Exp Eye Res. 2004 Mar;78(3):409-16. |
| Online Support for
Sjögren's |
| SWA
Invitation Do you have a Sjögren's web site? If
so, you are invited join the free international
Scleroderma Webmaster's Association. |
| Sjögren's
Support Email List (TalkSjo) provides support and sociability
for Sjögren's patients worldwide. Dry.Org |
| Sjögren's
Syndrome Medical Information List is a moderated list
subscribed to by hundreds of Sjögren's patients and
professionals worldwide. The main focus of the list is medical
information and anything that does not have to do specifically
with Sjögren's does not get posted. |
| Sjögren's
Email Support Lists on Yahoo Groups |
| Sjögren's Patient
Stories |
| Submit
your Sjögren's and/or Scleroderma Story (in any
language) for inclusion on Scleroderma from A to Z. It's
as easy as writing an email, using our simple online form. ISN |
| Annette
G: MCTD, Systemic Scleroderma, Sjogren's, Chronic Kidney
Disease, CFS, Fibromyalgia, Trigeminal Neuralgia, TMJ People
think that because I am a nurse I have all the answers. Well
guess what? I don't... |
| (Italiano)
Antea: Sindrome di Sjogren e il Lupus Eritematoso Sistemico Sono
Angela dalla provincia di pesaro, ho 29 anni e da ieri ho
scoperto di avere la Sindrome di Sjogren... |
| More Sjogren's Personal Stories... |
| Media Stories, Misc. |
| Women
with little-known disease work to raise awareness. Dana
Moreno and Connie Roberts have made it their mission to educate
doctors - and everyone else - about Sjogren's Syndrome, an
autoimmune deficiency that debilitates through myriad avenues:
the central nervous system first, then on to major organs
and the lymphatic system. St. Petersburg Times, Hernando.
03/22/07. |
| More
than just dry eyes. Sjogren's syndrome is often misdiagnosed.
It may occur alone or with a connective tissue disease, such
as rheumatoid arthritis, lupus or scleroderma. It affects
the entire body, ranging from mild to disabling. Deseret
Morning News. 03/04/07. |
| International
Group Tackles Sjögren's Syndrome (SS). The Sjögren’s
International Collaborative Clinical Alliance is working
toward establishing standardized diagnostic criteria for
SS and building an international registry of clinical data
and biospecimens that are available to scientists all over
the world. UCSF Today. 07/19/06. |
| Sjogren's
Syndrome - an often underdiagnosed condition. Sjogren's
syndrome occurs by itself or can and often does occur with
other autoimmune diseases such as lupus, rheumatoid arthritis,
scleroderma, and other lesser known rheumatic autoimmune
diseases. Irishhealth.com 01-04-06. |
