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$Unique_ID{BRK03877}
$Pretitle{}
$Title{IGA Nephropathy}
$Subject{IGA Nephropathy Berger's Disease Idiopathic Renal Hematuria Mesangial
IGA Nephropathy Schonlein-Henoch Purpura SLE Nephritis }
$Volume{}
$Log{}
Copyright (C) 1988, 1989 National Organization for Rare Disorders, Inc.
587:
IGA Nephropathy
** IMPORTANT **
It is possible that the main title of the article (IGA Nephropathy) is
not the name you expected. Please check the SYNONYM listing to find the
alternate names and disorder subdivisions covered by this article.
Synonyms
Berger's Disease
Idiopathic Renal Hematuria
Mesangial IGA Nephropathy
Information on the following diseases can be found in the Related
Disorders section of this report:
Schonlein-Henoch Purpura
SLE Nephritis
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.
IGA Nephropathy is a kidney disorder occuring during childhood and young
adulthood. It usually follows a viral infection of the upper respiratory or
gastrointestinal tracts. The major symptom is the passing of blood in the
urine (hematuria). There may be associated pain in the loin area.
Symptoms
The first recognizable symptom of IGA Nephropathy is bloody urine (hematuria)
caused by inflammation of the kidney (acute nephritis or glomerulonephritis).
There is often a mild loss of protein in the urine (proteinuria) with slowly
progressive changes in the kidney. Pain in the loins may occur, but it is
unusual for people with this condition to show signs of high blood pressure
(hypertension) or swelling (edema) during the initial phase of the disease.
Causes
IGA Nephropathy usually occurs following flu-like (viral) infections of the
upper respiratory tract or the gastrointestinal tract. This suggests it may
be caused by a postinfectious process. There are some theories that the
condition is an autoimmune disease because of the increase in the
immunoglobulin IGA factor, but the mechanisms leading to glomerular immune
deposit formation is unclear. Autoimmune disorders are caused when the
body's natural defenses (antibodies) against invading organisms suddenly
begin to attack healthy tissue.
Affected Population
IGA Nephropathy affects males two or three times more often than females. It
usually occurs in adolescents or young adults between the ages of fifteen and
thirty-five. It is one of the leading causes of acute nephritis in young
people in the United States, Europe and Japan. IGA Nephropathy occurs
significantly more often in American Indians than any other ethnic group
tested. It is more prevalent in whites than in blacks. A study conducted in
Finland showed an occurrence of approximately 94 cases detected annually per
100,000 young males tested upon induction into the military.
Related Disorders
Symptoms of the following disorders can be similar to those of IGA
Nephropathy. Comparisons may be useful for a differential diagnosis:
Schonlein-Henoch Purpura is one of a group of blood vessel disorders
characterized by purplish or brownish-red discoloration of the skin. These
spots may be large or small. Internal bleeding may occur in various areas of
the body. This blood vessel disorder may affect the skin, joints,
gastrointestinal system, kidneys, and in a very few cases, the central
nervous system. Little is known about the cause of this form of purpura
although it may be an allergic reaction which more often occurs in children
than in adults. Some cases of Schonlein-Henoch characterized by joint
disease without gastrointestinal problems are termed Schonlein's Purpura.
Another form, characterized by acute abdominal symptoms without joint disease
is known as Henoch's Purpura. This disorder runs a limited course with a
good prognosis in most cases. (For more information on this disorder, choose
"Purpura" as your search term in the Rare Disease Database).
SLE Nephritis is a kidney disease associated with Lupus. In severe cases
of Lupus, the kidneys are involved and may lead to kidney (renal) failure.
The following symptoms may occur in patients with Lupus: headache, swelling
of the eye area (periorbital edema) and swelling (edema) of the face,
abdomen, feet or legs, loss of appetite, weakness, development of excessive
fatigue upon exertion, malaise or weariness, mental and personality changes,
seizures, dizziness or fainting, pallor, shortness of breath, numbness of the
extremities, nausea, vomiting, diarrhea, and visual abnormalities. Tests
including urinalysis, blood serum BUN or creatinine, 24 hour creatinine
clearance and/or quantitative protein excretion are useful in detecting SLE
Nephritis. (For more information on this disorder, choose "Lupus" as your
search term in the Rare Disease Database).
Therapies: Standard
No specific treatment for IGA Nephropathy has been shown to be effective to
all patients. Some patients have responded to oral steroid therapy
especially in the early stages of the disease. Patients who have been
treated with the corticosteroid drug, cyclophosamide, have experienced long-
term remission of symptoms even after therapy was withdrawn. The disease may
progress slowly for several decades and can lead to other kidney (renal)
diseases such as renal insufficiency. Kidney transplantation has been
successful in many persons, although some patients have an immunologic
recurrence of disease in the new kidney.
Therapies: Investigational
The immunomodulator drug cyclosporin (sandimmune) may be an effective
treatment for certain patients with relapsing nephropathy. However, if the
drug is discontinued, most patients will relapse. Further studies will be
needed to determine the long-term effectiveness and safety of this drug in
treating Nephropathy.
This disease entry is based upon medical information available through June
1989. 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 IGA Nephropathy, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
IGA Nephropathy Support Network
234 Summit Ave.
Jenkintown, PA 19046
(215) 884-9038
National Kidney Foundation
2 Park Avenue
New York, NY 10016
(212) 889-2210
(800) 662-9010
American Kidney Fund
6110 Executive Blvd., Suite 1010
Rockville, MD 20852
(301) 881-3052
(800) 628-8299
(800) 492-8361 (MD)
National Kidney and Urologic Diseases Information Clearinghouse
Box NKUDIC
Bethesda, MD 20892
(301) 468-6345
References
INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and
Co., 1987. Pp. 841.
STEROID THERAPY IN IGA NEPHROPATHY: A RETROSPECTIVE STUDY IN HEAVY
PROTEINURIC CASES. Y. Kobayaski, et al.; Nephron (1988, issue 48 (1)). Pp.
12-17.
TONSILLAR DISTRIBUTION OF IGA AND IGG IMMUNOCYTES AND PRODUCTION OF IGA
SUBCLASSES AND J CHAIN IN TONSILLITIS VARY WITH THE PRESENCE OR ABSENCE OF IGA
NEPHROPATHY. J. Nagy, et al.; Scand J Immunol (April, 1988, issue 27 (4)).
Pp. 393-399.
PROTEINURIA IN IGA NEPHROPATHY. K. Neelakantappa, et al.; Kidney Int
(March, 1988, issue 33 (3)). Pp. 716-721.
IGA NEPHROPATHY, THE MOST COMMON GLOMERULONEPHRITIS WORLDWIDE. A
NEGLECTED DISEASE IN THE UNITED STATES? Am J Med (January, 1988, issue 84
(1)). Pp. 129-132.
CYCLOSPORIN IN THE TREATMENT OF STEROID-RESPONSIVE AND STEROID RESISTANT
NEPHROTIC SYNDROME IN ADULTS. E. Maher, et al.; NEPHROL DIAL TRANSPLANT,
(1988; 3 (6)).