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$Title{Felty Syndrome}
$Subject{Felty Syndrome Splenomegaly with Rheumatoid Arthritis}
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Copyright (C) 1987, 1989 National Organization for Rare Disorders, Inc.

342:
Felty Syndrome

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

Synonyms

     Splenomegaly with Rheumatoid Arthritis

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

     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.


Felty Syndrome is a rare form of Rheumatoid Arthritis which affects the
skin and spleen as well as the joints.  White blood cell abnormalities can
lead to associated infections.  Symptoms may improve with removal of the
spleen (splenectomy).  Felty Syndrome seems to occur mostly in middle-aged
women.

Symptoms

Symptoms of Felty Syndrome include a decrease in the white blood cells which
are linked to frequent infections; blood platelet reduction; joint stiffness
and/or aching (similar to Rheumatoid Arthritis);  enlargement of the spleen;
and a yellowish-brown discoloration of the skin.  Exposed areas of the skin
may also have symptoms resembling those of Pellagra (rough, scaly, reddish-
brown and stinging sensations).  Cell death in soft tissue of the mouth
(Ulceronecrotic Stomatitis), anemia, leg ulcers, possible swelling of lymph
nodes and fever may also occur with Felty Syndrome.

Causes

The exact cause of Felty Syndrome is not clear at this time.  Scientists
believe that the blood cell abnormalities, an allergy, or some unknown
immunity disturbance may lead to the frequent infections which are common
with this disorder.

Affected Population

Felty Syndrome seems to occur mostly in middle-aged women, although it has
been reported in adolescents as well.

Related Disorders

Rheumatoid Arthritis is a prevalent disorder that usually occurs in middle-
aged and older people, mostly women.  In rare instances Rheumatoid Arthritis
can affect children.  Pain, stiffness and deformities of joints are the main
symptoms of Rheumatoid Arthritis.  The hands, knees, feet, jaw, and the spine
are the areas most commonly affected.  (For more information on this
disorder, see the Arthritis section of the Prevalent Health
Conditions/Concerns area of NORD Services.)

Although the exact cause of Rheumatoid Arthritis is not known, it is
suspected to be an autoimmune disorder.  Autoimmune disorders are caused when
the body's natural defenses against invading organisms (antibodies), for
unknown reasons, suddenly begin to attack healthy tissue.

Therapies:  Standard

The most common treatment for serious and recurrent infections which are
caused by Felty Syndrome is removal of the spleen (splenectomy).  This
treatment is successful in about fifty percent of cases.  Other treatments
are those used for Rheumatoid Arthritis such as anti-inflammatory drugs, gold
salts or penicillamine.  Anemia associated with Felty Syndrome can be treated
with blood transfusions.  The prognosis is generally uncertain and depends
upon several variables including the general health of the patient and the
combination of symptoms occurring in a patient.

Therapies:  Investigational

Low dose oral prednisone therapy has been used experimentally to treat Felty
Syndrome.  Preliminary studies appear positive, but more research is needed.

This disease entry is based upon medical information available through
March 1987.  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 Felty Syndrome, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     (203) 746-6518

     Arthritis Foundation
     1314 Spring Street NW
     Atlanta, GA  30309
     (404) 872-7100

     The National Arthritis and Musculoskeletal and Skin Diseases Information
     Clearinghouse
     Box AMS
     Bethesda, MD  20892
     (301) 495-4484

References

FELTY'S SYNDROME IN A CHILD:  A.M. Rosenberg, et. al.;  J Rheumatol (December
1984, issue 11(6) ).  Pp. 835-837.