$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)).