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$Unique_ID{BRK04224}
$Pretitle{}
$Title{Sjogren Syndrome}
$Subject{Sjogren Syndrome Dacryosialoadenopathia atrophicans
Gougerot-Houwer-Sjogren Gougerot-Sjogren Keratoconjunctivitis Sicca
Keratoconjunctivitis sicca-xerostomia Secreto-inhibitor-xerodermostenosis
Sicca Syndrome Xerostomia Mikulicz Syndrome Fibromyalgia Keratomalacia
Ligneous Conjunctivitis Lupus (Systemic Lupus Erythematosus or SLE) Rheumatoid
Arthritis}
$Volume{}
$Log{}

Copyright (C) 1992 National Organization for Rare Disorders, Inc.

6:
Sjogren Syndrome

** IMPORTANT **
It is possible the main title of the article (Sjogren Syndrome) is not
the name you expected.  Please check the SYNONYMS listing on the next page to
find alternate names and disorder subdivisions covered by this article.

Synonyms

     Dacryosialoadenopathia atrophicans
     Gougerot-Houwer-Sjogren
     Gougerot-Sjogren
     Keratoconjunctivitis Sicca
     Keratoconjunctivitis sicca-xerostomia
     Secreto-inhibitor-xerodermostenosis
     Sicca Syndrome
     Xerostomia

Information on the following diseases can be found in the Related
Disorders section of this report:

     Mikulicz Syndrome
     Fibromyalgia
     Keratomalacia
     Ligneous Conjunctivitis
     Lupus (Systemic Lupus Erythematosus or SLE)
     Rheumatoid Arthritis

General Discussion

** REMINDER **
The Information contained in the Rare Disease Database is provided for
educational purposes only.  It should not be used for diagnostic or treatment
purposes.  If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.

Sjogren syndrome is an autoimmune disorder characterized by degeneration
of the mucous-secreting glands, particularly the tear ducts of the eyes
(lacrimal) and saliva glands of the mouth.  Autoimmune disorders are caused
when the body's natural defenses (antibodies, lymphocytes, etc.), against
invading organisms suddenly begin to attack healthy tissue.  It is also
associated with inflammatory disorders such as arthritis or Lupus.

Symptoms

Sjogren Syndrome generally has a sudden onset.  Primary Sjogren Syndrome is
characterized by inflammation of the cornea of the eyes and the delicate
membranes that line the eyelids (keratoconjunctivitis) due to insufficient
tear production, and dryness of the mouth (sicca xerostemia) due to lack of
saliva from the salivary glands.   In secondary Sjogren syndrome, dry eyes
and/or mouth may occur with diseases of the tissue that holds together and
supports different structures of the body (connective tissue disease).  Most
often rheumatoid arthritis (RA), Lupus or other autoimmune diseases are
present with secondary Sjogren syndrome.

Most patients with Sjogren syndrome have the primary type of Sjogren
syndrome.  The onset of symptoms is usually sudden.  Decreased production of
saliva and the resulting dry mouth make chewing and swallowing food
difficult.  The lack of saliva causes pieces of food to stick to the cheeks,
gums and throat.  Teeth decay easily, leading to cavities (dental caries),
inflammation of the gums (gingivitis) and advanced gum disease (pyorrhea).

As the tear ducts of the eyes (lacrimal glands) waste away (atrophy), the
amount of tears produced decreases, causing a feeling of grittiness and
burning in the eyes.  The eyelids may stick together, glands under the jaw
may be swollen and painful, and gastrointestinal symptoms may occur.

Dryness may extend to the skin and to the mucous membranes lining the
nose, throat and vagina.  Muscle pain and weakness may also occur
(Fibromyalgia).

In secondary Sjogren syndrome, patients may experience arthritis, rash
(palpable purpura) on the lower extremities, and light sensitive rashes
(photosensitive dermatitis) on the face, arms and other exposed areas.  Fever
and neurologic symptoms may occur.

Patients with systemic Sjogren Syndrome (symptoms in addition to the eyes
and mouth) usually have blood tests that are positive for certain antibodies
(anti-nuclear antibodies to Ro and La antigens).  Antibodies are substances
made by the body that defend the body against bacteria, viruses, or other
foreign invaders (antigens).

All patients suspected of having Sjogren syndrome should be examined by
an ophthalmologist, a physician who specializes in the care and treatment of
eyes.  Patients with Sjogren Syndrome who have positive blood tests for anti-
Ro antibodies should be evaluated by a physician who specializes in the care
and treatment of inflammatory diseases (rheumatologist) for evidence of
disease outside of the eyes and mouth (extra-glandular involvement).

Causes

Sjogren syndrome is an autoimmune disorder.  It has no known cause.
Autoimmune disorders are caused when the body's natural defenses (antibodies,
lymphocytes, etc.), against invading organisms suddenly begin to attack
healthy tissue.

People with Sjogren syndrome often have a genetic predisposition (HLA-
DR3).  A genetic predisposition means that a person may carry a gene for a
disease but it may not be expressed unless something in the environment
triggers the disease.  Secondary Sjogren syndrome often occurs in patients
with rheumatoid arthritis, systemic lupus erythematosus and other connective
tissue diseases.

Affected Population

Sjogren syndrome affects 9 females to every male.  Ninety percent of women
with the disorder have already gone through menopause (post-menopausal),
although symptoms may be apparent at an earlier age.  Recent data suggests
that men who show symptoms of HIV infection may develop a syndrome similar to
Sjogren's.

Related Disorders

Symptoms of the following disorders can be similar to those of Sjogren
Syndrome.  Comparisons may be useful for a differential diagnosis:

Mikulicz Syndrome is a benign chronic disorder that causes the
enlargement of the tonsils, the saliva glands located near the ear (parotid),
the glands beneath the upper jaw bone (submaxillary), glands that produce
tears (lacrimal glands), and the salivary glands of the mouth.  Symptoms may
include dryness of the mouth, difficulty swallowing and tooth decay.  There
may be absent or decreased tears and blurred vision.  (For more information
on this disorder, choose "Mikulicz Syndrome" as your search term ion the Rare
Disease Database).

Keratomalacia is an eye disease caused by a deficiency of vitamin A.
Vitamin A (retinol) is found mainly in fish liver oils, liver, egg yolk,
cream and butter.  The human body stores vitamin A mainly in the liver.  Once
it is released by the liver, vitamin A is converted to light sensitive
pigments in the retina of the eye which is involved in night, day and color
vision.  Vitamin A also helps to maintain healthy body tissues.  A lack of
Vitamin A may cause night blindness, abnormal dryness of the inner surface of
the eyelid (xerosis) and the transparent covering of the eyes (cornea).  This
dryness may result in a feeling of grittiness in the eyes and a painful
sensitivity to light (photophobia).  (For more information on this disorder,
choose "Keratomalacia" as your search term in the Rare Disease Database).

Ligneous Conjunctivitis is a rare disorder that is characterized by
lesions in the mucous producing membranes especially of the eye.  This
disorder usually presents itself in childhood.  Mucous membranes of the voice
box (larynx), vocal chords, nose, trachea, wind pipe, vagina, cervix and gums
(gingiva) may also be affected.  The lesions in the membranes have a "wood-
like" (ligneous) texture.  They are thick, firm, knotty and tough.  The cause
of this disorder is not known although there have been some cases that
suggest an autosomal recessive genetic inheritance.  (For more information on
this disorder, choose "Ligneous Conjunctivitis" as your search term in the
Rare Disease Database).

Fibromyalgia is a chronic muscle disorder characterized by muscle pain
throughout much of the body.  This may begin gradually or have a sudden
onset.  Other symptoms include muscle spasms, fatigue, muscle tissue
stiffness and unrefreshing (non-restorative) sleep.  The exact cause of this
disorder is not known.  Some patients with Fibromyalgia may have chest pain,
headaches, diarrhea, constipation, dryness in the eyes and mouth (Sjogren
syndrome), swelling of a tendon (tendinitis), or swelling of the connective
tissue surrounding a joint (bursitis).  (For more information on this
disorder, choose "Fibromyalgia" as your search term in the Rare Disease
Database).

Lupus (Systemic Lupus Erythematous or SLE) is a multi-system inflammatory
disease of the connective tissue of the body.  Sjogren syndrome may occur in
conjunction with Lupus.  Fatigue is an early and frequent symptom.  Other
symptoms may include fever, swollen glands, skin rash, profound weight loss,
headaches, hair loss (alopecia) and water retention (edema).  Arthritis,
joint and muscle pain occurs in 90 percent of the cases.  These symptoms may
occur years before the illness is actually diagnosed.  The arthritis symptoms
come and go and tend to appear most often in either knees, fingers, or wrist
joints.  There is no bone loss associated with this joint involvement.  (For
more information on this disorder, choose "Lupus" as your search term in the
Rare Disease Database).

Rheumatoid Arthritis (RA) is an autoimmune inflammatory disorder.  It's
cause is unknown.  It is characterized by morning stiffness and arthritis
mainly in the hands, wrists, knees, feet, shoulders and hips.  Once a joint
is involved, it may remain painful for weeks, months, and even years.  About
25 percent of RA patients also have Sjogren syndrome (secondary).  (For more
information on this disorder, choose "Rheumatoid Arthritis as your search
term in the Rare Disease Database).

Therapies:  Standard

A number of tests are available for the diagnosis of Sjogren syndrome.  They
include a careful examination of the eyes, including the measurement of tear
production;  measurement of saliva production after stimulation with lemon
juice; X-ray of the glands under the jaw and ears (parotid glands);
examination of the cells of the lip to determine if a special type of small
white blood cells (lymphocytes) are present in the salivary glands (biopsy);
blood tests (including ANA anti-nuclear antibody and Immunoglobulin levels or
Ig levels).  An ophthalmologist or a rheumatologist should be contacted for
testing.

Treatment of Sjogren syndrome is dependent on symptoms and usually is
much the same as for other autoimmune disorders.  No treatment, however, has
yet been found to restore the secretions of the glands involved.  The
insufficient secretions can be replaced by artificial tears in the form of
eye drops, artificial saliva which can be used to wet the mouth, and vaginal
lubricants.  Medications such as corticosteroids, anti-inflammatory drugs or
cytoxan may be needed for certain complications.

Therapies:  Investigational

Medical research is seeking to determine the exact cause of this disorder, as
well as development of new treatments.

The National Institute of Dental Research (NIDR) is conducting studies on
several drugs for treatment of Sjogren Syndrome.  Patients interested in
participating in these studies should ask their physician to contact:

     Alice Macynski, RN
     NIH/National Institute of Dental Research (NIDR)
     9000 Rockville Pike
     Bldg. 10, Rm. 1B-21
     Bethesda, MD  20892
     [phone]

Bromhexine is a drug used in Europe and Canada for the treatment of
Sjogren syndrome.  However no clinical trials are underway in the United
States.

Trials of the drug Pilocarpine for treatment of dry mouth has been
suggested by researchers.  The drug increases the salivary flow rate in test
subjects.  The immune suppressive drug, Cyclosporine, is being developed as a
special formulation for use as an eye medication with the hope that it may
reduce destruction of tear ducts in Sjogren Syndrome.   As with any drug,
more study is needed to determine the long-term safety and effectiveness of
these experimental treatments.

This disease entry is based upon medical information available through
August 1992.  Since NORD's resources are limited, it is not possible to keep
every entry in the Rare Disease Database completely current and accurate.
Please check with the agencies listed in the Resources section for the most
current information about this disorder.

Resources

For more information on Sjogren Syndrome, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT 06812-1783
     [phone]

     Sjogren Syndrome Foundation
     382 Main St.
     Port Washington, NY 11050
     [phone]

     National Sjogren Syndrome Association
     3201 W. Evans Dr.
     Phoenix, AZ  85023
     [phone]
     [phone]

     The Arthritis Foundation
     1314 Spring Street NW
     Atlanta, GA 30309
     [phone]

     NIH/National Institute of Dental Research (NIDR)
     9000 Rockville Pike
     Bethesda, MD 20392
     [phone]

     NIH/National Arthritis and Musculoskeletal and Skin Diseases Information
Clearinghouse
     Box AMS
     Bethesda, MD 20892
     [phone]

References

MENDELIAN INHERITANCE IN MAN, 9th Ed.:  Victor A. McKusick, Editor:  Johns
Hopkins University Press, 1990.   Pp. 1477-1478.

CECIL TEXTBOOK OF MEDICINE, 19th Ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Editors; W.B. Saunders Co., 1990.  Pp. 1535-1537.

PRIMARY SJOGREN'S SYNDROME IN MEN.  CLINICAL SEROLOGIC, AND IMMUNOGENETIC
FEATURES.  R. Molina, et al.; Am J Med, (January, 1986, issue 80(1)).  Pp.
23-31.

TREATMENT OF PRIMARY SJOGREN'S SYNDROME WITH HYDROCHLOROQUINE. R.I. Fox,
et al; Am J Med (October 14, 1988, issue 85 (4A)).  Pp 62-67.

MOLECULAR CHARACTERIZATION OF A MAJOR AUTO-ANTIBODY ASSOCIATED CROSS-
REACTIVE IDIOTYPE IN SJOGREN'S SYNDROME.  T.J. Kipps, et al.; J Immunol (June
15, 1989, issue 142 (12)).  Pp. 4261-4268.