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$Title{Lupus}
$Subject{Lupus Systemic Lupus Erythematosus SLE Lupus Erythematosus
Disseminated Lupus Erythematosus Scleroderma Polymyositis Dermatomyositis
Osteoarthritis Sjogren's Syndrome Mixed Connective Tissue Disease (MCTD)
Raynaud's Disease and Phenomenon}
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Copyright (C) 1984, 1985, 1987, 1988, 1990, 1992, 1993 National
Organization for Rare Disorders, Inc.

38:
Lupus

** IMPORTANT **
It is possible that the main title of the article (Lupus) is not the name
you expected.  Please check the SYNONYMS listing to find the alternate name
and disorder subdivisions covered by this article.

Synonyms

     Systemic Lupus Erythematosus
     SLE
     Lupus Erythematosus
     Disseminated Lupus Erythematosus

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

     Scleroderma
     Polymyositis
     Dermatomyositis
     Osteoarthritis
     Sjoren's Syndrome
     Mixed Connective Tissue Disease (MCTD)
     Raynaud's Disease and Phenomenon

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.


Lupus (Systemic Lupus Erythematosus) is an inflammatory disease of the
connective tissue.  Tissue damage occurs when the body's own immune system
attacks healthy tissue causing inflammation and malfunction of various organ
systems.  Many different symptoms are associated with Lupus, and most
patients do not get all of the symptoms.

Symptoms

Lupus is an inflammatory disease of the connective tissue.  The initial
symptom of Lupus is usually excessive fatigue.  Other early symptoms may
include fever, swollen glands, loss of appetite (anorexia), weight loss,
headaches, loss of hair (alopecia), and swelling due to fluid retention
(edema).

Over 90 percent of people with Lupus experience inflammation and swelling
of joints (arthritis), joint pain (arthralgia), and generalized muscle pain
(myalgia).  The knees, fingers, and wrist joints are the most likely to be
affected by arthritis-like pain associated with Lupus.  In some cases these
arthritis symptoms may precede the onset of Lupus by months or even years.
Frequently joints on both sides of the body are affected.  The inflammation
and joint pain associated with Lupus often moves from one area of the body to
another, and generally does not destroy the cartilage or bones within the
joints (non-erosive).

Over 80 percent of people with Lupus experience dermatological (skin)
problems.  Light sensitive (photosensitive) rashes and other lesions may
include:  ring-shaped eruptions surrounded by a clear unaffected disk of skin
(annular lesion), scaly red spots (discoid lesions), and/or thin walled
blisters on the skin greater than one centimeter in diameter containing clear
fluid (bullae).  About 50 percent of people with Lupus will get a classic red
(erythematous) "butterfly rash" across the bridge of the nose and cheeks.
This rash may last for hours or days.  Lesions of the mucous membranes that
line the mouth and nose occur in about 35 percent of patients with Lupus.

Lupus may also affect the vascular (blood vessel) system.  Vascular
involvement may include:  a permanent increase in the diameter (dilation) of
very small blood vessels (capillary telangiectasia), painfully cold fingers
and toes caused by spasms of small vessels in response to cold (Raynaud's
phenomenon), and/or inflammation of the blood vessels (vasculitis).

Respiratory involvement may also occur with Lupus.  Symptoms may include
inflammation of the membranes (parietal pleura) that surround the lungs
(pleurisy), a persistent cough, and inflammation of the lungs (pneumonitis).
Lupus may also affect the heart.  Cardiac abnormalities may include:
inflammation of the membranous sac that surrounds the heart (pericarditis),
inflammation of the muscles of the walls of the heart (myocarditis), and/or
coronary artery disease.

Lupus may affect the blood and its various components (hematological
system).  Symptoms may include:  low levels of hemoglobin (anemia), an unusual
decrease in the number of white blood cells (leukopenia), a decrease in the
number of lymphocytes associated with the immune function of the body
(lymphocytopenia), a decrease in the number of platelets (thrombocytopenia),
and/or disorders of the lymph nodes or lymphatic vessels (lymphadenopathy).
These abnormalities of the blood often occur early in the course of Lupus.

People with advanced Lupus may sustain kidney and urinary system
problems.  Elevated levels of protein in the urine (proteinuria),
inflammation of the kidneys (interstitial nephritis), and inflammation of the
glomerulus (a cluster of blood vessels and nerve fibers) of the kidney
(glomerulonephritis), may occur.

Behavioral (neuropsychiatric) symptoms of Lupus may include depression,
anxiety or psychosis.  Seizures and stroke may also occur.  Other
neurological symptoms may include inflammation and degeneration of the nerve
fibers outside the brain and spinal cord (peripheral neuropathy), and
inflammation of the membrane that surrounds the brain and spinal cord
(meningitis).

A tentative diagnosis of Lupus can be made if 4 of the following criteria
are present:  arthritis involving 2 or more major joints, a rash on the
cheeks (malar rash), discoid rash, oral or nasal ulcers, photosensitivity,
pleuritis or pericarditis, positive LE cell test, presence of anti-DNA or
anti-Sm, or a chronic false positive blood test for syphilis, increased
levels of protein in the urine (proteinuria over 0.5 grams/day), cellular
casts in the urine, seizures or psychosis, hemolytic anemia, leukopenia,
lymphopenia, or thrombocytopenia, and/or an abnormal antinuclear antibody
blood test (titer).

People with Lupus sometimes experience a temporary flare-up or worsening
of symptoms.  Flare-ups may occur several times a year or once every few
years.  These episodes of severe symptoms may be triggered by such factors as
stress, infections and exposure to sunlight.

Causes

Lupus is an autoimmune disease of the connective tissue.  The cause of Lupus
is unknown.  Immunologic, genetic, environmental, hormonal and/or infectious
factors may be involved.  Autoimmune disorders are caused when the body's
natural immune defenses (antibodies, lymphocytes, etc.), against invading
organisms mistakenly attack healthy tissue.

Scientists suspect a genetic basis for Lupus.  Based on studies of twins,
researchers have found that one that even if one twin has Lupus and the other
is healthy, both twins manufacture abnormal antibodies.  However, the healthy
twin manufactures fewer antibodies than the twin with Lupus.  Scientists do
not yet understand the pattern of inheritance of the gene that makes people
susceptible to Lupus.

"Lupus-like" symptoms have also been induced by some drugs, including
hydralazine, procainamide, isoniazid, methyldopa and chlorpromazine.

Affected Population

Lupus primarily affects females.  Ninety percent of the cases of Lupus occur
in women of any age although it commonly begins between the ages of 15 to 55
years.  African American women are 3 times more likely to get Lupus than
Caucasian women.   Lupus is also commonly seen in Asian women.  Estimates of
the prevalence of Lupus in the United States vary considerably.  However, the
most reliable estimate is that Lupus affects approximately 50 in 100,000
people in the United States.

Related Disorders

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

Scleroderma is a rare connective tissue disorder.  The cause is unknown.
Scleroderma is characterized by skin thickening, painfully cold fingers and
toes caused by spasms of small vessels in response to cold (Raynaud's
phenomenon), and a wide range of multi-system disorders.  The early symptoms
of Scleroderma may include:  skin rashes, joint pain, morning stiffness,
fatigue, and/or weight loss.  As the disease progresses the skin becomes
rigid, dry, and smooth and may be yellowish or ivory-colored (morphea).  (For
more information on this disorder, choose "Scleroderma" as your search term
in the Rare Disease Database).

Polymyositis is a rare connective tissue disease.  The cause is unknown.
Polymyositis is characterized by inflammatory degenerative changes in the
muscle fibers and the supportive collegen of connective tissue.  The major
early symptom of this disorder is muscle weakness usually in the neck, trunk
and shoulders.  Eventually it may become difficult to rise from a sitting
position, climb stairs, lift objects and/or reach overhead.  Occasionally,
joint pain and tenderness also occur.  Other symptoms may also include:
inflammation of the lungs (interstitial pneumonitis), difficulty breathing,
coughing, painfully cold fingers in response to cold (Raynaud's phenomenon),
digestive problems, and/or heart irregularities.  (For more information on
this disorder, choose "Polymyositis" as your search term in the Rare Disease
Database).

Dermatomyositis is a rare connective tissue disease.  The cause is
unknown.  Dermatomyositis is identical to Polymyositis but with the addition
of a characteristic red skin rash.  These red rashes generally occur before
the muscle weakness and usually appear on the face, knees, shoulders and
hands.  In some patients the skin changes caused by Dermatomyositis are
similar to those of Scleroderma.  The skin may become dry, hard and have a
brownish color.  (For more information on this disorder, choose
"Dermatomyositis" as your search term in the Rare Disease Database).

Osteoarthritis is a common autoimmune disease in which one or many joints
undergo degenerative changes.  Osteoarthritis is characterized by the loss of
cartilage, deformities of bones and joints, and the thickening of the
surrounding ligaments and membranes.  Osteoarthritis develops when joint
repair does not keep pace with bone degeneration.  It may also occur as a
result of trauma to the bone, aging, obesity, or other underlying disease.
Symptoms include pain and joint stiffness particularly in the morning.

Sjogren's Syndrome is a rare autoimmune disorder that is characterized by
the degeneration of mucous-secreting glands, particularly those of the eyes
and mouth.  Sjogren's Syndrome can also be associated with rheumatoid
arthritis and Lupus.  The major symptoms of this disorder include a dry mouth
(xerostomia) caused by decreased production of saliva, and dry eyes caused by
decreased production of tears.  Sjogren's Syndrome primarily affects women,
and it often includes muscle pain and arthritis along with mucous membrane
symptoms.  (For more information on this disorder, choose "Sjogren" as your
search term in the Rare Disease Database).

Mixed Connective Tissue Disease (MCTD) is a rare inflammatory disorder of
the connective tissue.  The symptoms of this disorder overlap with those of
Lupus (Systemic Lupus Erythematosus), scleroderma and polymyositis.  Early
symptoms may include:  a fever of unknown origin, painfully cold fingers in
response to cold (Raynaud Phenomenon), swollen hands, fatigue, and/or non-
deforming arthritis.  Arthritis occurs in almost every case of MCTD, but
rarely results in deformities similar to those seen in Rheumatoid Arthritis.
Muscle pain and skin rashes are also very common.  These rashes may be
similar to those in people with Lupus.  (For more information on this
disorder, choose "Mixed Connective Tissue Disease" as your search term in the
Rare Disease Database.)

Raynaud's Disease is a rare disorder characterized by spasms of the blood
vessels in the fingers and skin and is considered to be a benign form of
Raynaud's Phenomenon which occurs along with other systemic disorders.  The
major symptom of this disorder is a dramatic stark white pallor of the
affected fingers and toes when exposed to cold, although a blue or red color
may also be present from time to time.  Other symptoms in the affected
fingers and toes vary in response to cold and may include:  a feeling of
numbness or cold, severe aching or pain, tingling or throbbing, a sensation
of tightness, "pins and needles," and/or a profound loss of sensation.  (For
more information on this disorder, choose "Raynaud" as your search term in
the Rare Disease Database.)

Therapies:  Standard

The symptoms of Lupus such as joint pain and fever commonly respond to
aspirin or other nonsteroidal anti-inflammatory drugs.  Anti-malarial drugs
such as hydorxychloroquine and chloroquine may treat skin rashes effectively.
Precaution should be used when taking anti-malarial medications.  Prolonged
treatment with these drugs may cause side effects such as visual disturbances
and nausea.

The standard treatment for the more severe symptoms of Lupus is the
administration of corticosteroid drugs.  Prednisone or its equivalent are the
most frequently used drugs in this category.  Initial treatment and
maintenance dosages vary according to what organ system or systems are
involved, the patient's response to these medications, possible side effects
and duration of use.

Corticosteroid creams and lotions may effectively control some rashes and
skin irritation that are caused by Lupus.  These creams should be used with
caution on the face and in the presence of skin infection.  Since infections
can be a major problem for people with Lupus, any infection should be treated
immediately and aggressively with antibiotics.

People with Lupus should avoid over-exposure to ultraviolet light.  This
includes exposure to direct sunlight.  They should also avoid the use of oral
contraceptives.  For Lupus patients who experience severe kidney damage,
hemodialysis may be prescribed when kidney function is lost.

Therapies:  Investigational

Immunosuppressive therapies (drugs that suppress the immune system) for the
treatment of people with Lupus are sometimes used in cases where the kidneys
are involved.  It is thought that suppression of the immune system will also
suppress the formation of the antibodies which appears to cause the
widespread destruction of tissue characteristic of Lupus.

An important side effect of immunosuppressive drugs is an increased
susceptibility to infections.  However, some common immunosuppressive drugs
that are sometimes used to treat Lupus include azathioprine and
cyclophosphamide in combination with corticosteroids.  Deflazort is an anti-
inflammatory cortico-derivative drug that may cause less bone loss than
prednisone with equal effectiveness.  Research is underway to determine the
possible long-term side effects and effectiveness of Deflazort for use as a
treatment for Lupus.

Researchers are studying the use of Cyclophosphamide for people with
Lupus who have kidney or central nervous system involvement.
Cyclophosphamide is a powerful drug used to treat certain types of cancer.
Intravenous cyclophosphamide given once per month may enable people who have
Lupus to significantly reduce their daily doses of prednisone.  Further
research is needed on this treatment.

Studies are being conducted on the use of immune globulin as an
intravenous treatment for Lupus.  Further investigation is needed to
determine its safety and effectiveness.

Other experimental treatments include corticosteroids given intravenously
and attempts at plasmapheresis to physically remove the damaging combinations
of antibodies and associated proteins from the circulating blood of people
with Lupus.   Plasmapheresis is a method for removing unwanted substances (in
this case antibodies and associated proteins) from the blood.  Blood is
removed from the patient and blood cells are separated from plasma.  The
patient's plasma is then replaced with other human plasma and the blood is
transfused back into the patient.  More research is needed before
plasmapheresis can be recommended for use in all but the most severe cases of
Lupus.

Lymphoplasmapheresis (a similar procedure involving lymphocytes) is also
being investigated as a possible treatment for people with advanced Lupus.

An orphan drug is being tested in the treatment of Lupus Nephritis.  The
drug is monoclonal antibody for immunization against this type of nephritis.
The drug is sponsored by:  Medclone, Inc., 2435 Military Ave., Los Angeles,
CA, 90064.

This disease entry is based upon medical information available through
February 1993.  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 Lupus, please contact:

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

     Lupus Foundation of America, Inc.
     4 Research Place, Suite 180
     Rockville, MD  20850
     [phone]
     [phone]

     Lupus Foundation of America, Inc.
     P.O. Box 2446
     Victorville, CA  92393

     Systemic Lupus Erythematosus Foundation, Inc.
     95 Madison Avenue, Room 1402
     New York, NY  10016
     [phone]

     The American Lupus Society
     3914 Del Amo Blvd., Suite 922
     Torrance, Ca  90503
     [phone]
     [phone]

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

References

THE MERCK MANUAL 15th ed:  R. Berkow, et al:  eds; Merck, Sharp & Dohme
Research Laboratories, 1987.  P. 1274.

MENDELIAN INHERITANCE IN MAN, 10th Ed.:  Victor A. McKusick, Editor:
Johns Hopkins University Press, 1992.  Pp. 675-676.

CECIL TEXTBOOK OF MEDICINE, 19th Ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Editors; W.B. Saunders Co., 1990.  Pp. 1522-1530.

SYSTEMIC LUPUS ERYTHEMATOUS, D.S. Pisetsky; Curr Opin Immunol (Dec 1991
3(6)): Pp. 917-923.

TREATMENT OF SYSTEMIC LUPUS ERYTHEMATOSUS, M.L. Miller; Curr Opin
Rheumatol (Oct 1991 3(5)):  Pp. 803-808.

SYSTEMIC LUPUS ERYTHEMATOUS, M.C. Hochberg; Rheum Dis Clin North Am (Aug
1990 16(3)):  Pp. 617-639.

ARTHRITIS AND RHEUMATISM:  M. Reichlin, et al.; Arthritis and Rheumatism
(April, 1992, issue 35 (4)).  Pp. 457-64.