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IgA Nephropathy

Synonyms of IgA Nephropathy

  • Berger's Disease
  • Idiopathic Renal Hematuria
  • Mesangial IGA Nephropathy

Disorder Subdivisions

  • No subdivisions found.

General Discussion

IgA nephropathy is a chronic kidney disease that usually first appears during adolescence and young adulthood and often progresses to kidney failure. 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 against "foreign" or invading organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons.

Affected Populations

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 believed to affect as many as 130,000 people annually in the United States, and 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 Americans of European descent than in Americans of African descent. 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:

Henoch-Shonlein 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 Henoch-Shonlein 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 "Henoch-Shonlein 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.)

Standard Therapies

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.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

On Dec 3rd of 2007 BioMarin Pharmaceuticals and IGAN Biosciences announced a collaboration to initiate a program to develop an IgA protease for treating IgA Nephropathy. IgA protease is shown to cleave the IgA complex which can casue the nephropathy.

BioMarin has executed a research and option agreement with IGAN Bioseciences for intellectual property covering IgA protease for treating IgA nephropathy.

For more information on both companies and further details please visit their website

IGAN Biosciences- www.iganbio.com
BioMarin Pharmceutical Inc-www.BMRN.com

CuraGen Corporation of New Haven, CT, announced on November 11, 2004, that its drug, CR002, a human monoclonal antibody, was granted FDA orphan drug status as a potential treatment to slow the progression of IgA nephropathy and delay kidney failure in patients affected by the disease. The drug is in a Phase I trial. For information, contact the company at:

CuraGen Corporation
555 Long Wharf Drive
New Haven, CT 06511
Tel.: (888) GENOMICS
Fax: (203) 401-3331
Email: info@curagen.com
Web: www.curagen.com

Omega-3 (n-3) polyunsaturated fatty acids (Omacor) received orphan product designation for treatment of IgA nephropathy in May, 2000. The drug is produced by Pronova Biocare, PO Box 420, 1327 Lysaker, Norway.

IgA Nephropathy Resources

Organizations:

References

TEXTBOOKS
Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:1863-64.

Larson DE. ed. Mayo Clinic Family Health Book. New York, NY: William Morrow and Company, Inc; 1996:838.

Bennett JC, Plum F. Eds. Cecil Textbook of Medicine. 20th ed. W.B. Saunders Co., Philadelphia, PA; 1996:575-76.

REVIEW ARTICLES
Goumenos DS, Brown CB. Therapeutic approach of patients with IgA nephropathy. Ren Fail. 2004;26:171-77.

Julian BA, Novak J. IgA nephropathy: an update. Curr Opin Nephrol Hypertens. 2004;13:171-79.

Delos Santos NM, Wyatt RJ. Pediatric IgA nephropathies: clinical aspects and therapeutic approaches. Semin Nephrol. 2004;24:269-86.

Xie Y, Chen X, Nishi S, et al. Relationship between tonsils and IgA nephropathy as well as indications for tonsillectomy. Kidney Int. 2004;65:1135-44.

Wada J, Sugiyama H, Makino H. Pathogenesis of IgA nephropathy. Semin Nephrol. 2003;23:556-63.

Samuels JA, Strippoli GF, Craig JC, et al. Immunosuppressive agents for treating IgA nephropathy. Cochrane Database Syst Rev. 2003;(4):CD003965.

Cattran DC. Outcomes research in glomerulonephritis. Semin Nephrol. 2003;23:340-54.

Strippoli GF, Manno C, Schena FP. An evidence-based survey of therapeutic options for IgA nephropathy: assessment and criticism. Am J Kidney Dis. 2003;41:1129-39.

Scolari F. Inherited forms of IgA nephropathy. J Nephrol. 2003;16:317-20.

FROM THE INTERNET
McKusick VA, ed. Online Mendelian Inheritance In Man (OMIM). The Johns Hopkins University. IgA Nephropathy. Entry Number; 161950: Last Edit Date; 5/24/2004.

IgA Nephropathy. National Kidney and Urologic Diseases Clearinghouse (NKUDIC). August 2003. 4pp.
http://kidney.niddk.nih.gov/kudiseases/pubs/iganephropathy/

IgA Nephropathy. National Kidney Federation (nkf). Last updated: 10 January 2001. 3pp.
www.kidney.org.uk/Medical-Info/kidney-disease/Iga.html

IgA Nephropathy. National Kidney Foundation. AtoZ GUIDE. 4pp.
www.kidney.org/atoz/atozItem.cfm?id=76

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Report last updated: 2007/12/05 00:00:00 GMT+0

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