IgA Nephropathy

IgA Nephropathy

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report IgA Nephropathy is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

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

Disorder Subdivisions

  • None

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.

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.

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

Resources

American Kidney Fund, Inc.

11921 Rockville Pike

Suite 300

Rockville, MD 20852

USA

Tel: (800)638-8299

Email: helpline@kidneyfund.org

Internet: http://www.kidneyfund.org



IgA Nephropathy Support Network

89 Ashfield Road

Shelburne Falls, MA 01370

USA

Tel: (413)625-9339

Internet: http://www.igansupport.org



National Kidney Foundation

30 East 33rd Street

New York, NY 10016

Tel: (212)889-2210

Fax: (212)689-9261

Tel: (800)622-9010

Email: info@kidney.org

Internet: http://www.kidney.org



Urology Care Foundation

1000 Corporate Blvd

Linthicum, MD 21090

USA

Tel: (410)689-3700

Fax: (410)689-3896

Tel: (800)828-7866

Email: Info@UrologyCareFoundation.org

Internet: http://www.urologyhealth.org/



NIH/National Kidney and Urologic Diseases Information Clearinghouse

3 Information Way

Bethesda, MD 20892-3580

Fax: (703)738-4929

Tel: (800)891-5390

TDD: (866)569-1162

Email: nkudic@info.niddk.nih.gov

Internet: http://www.kidney.niddk.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



Autoimmune Information Network, Inc.

PO Box 4121

Brick, NJ 08723

Fax: (732)543-7285

Email: autoimmunehelp@aol.com



European Society for Immunodeficiencies

1-3 rue de Chantepoulet

Geneva, CH 1211

Switzerland

Tel: 410229080484

Fax: 41229069140

Email: esid@kenes.com

Internet: http://www.esid.org



Kidney & Urology Foundation of America, Inc.

2 West 47th Street

Suite 401

New York, NY 10036

Tel: (212)629-9770

Fax: (212)629-5652

Tel: (800)633-6628

Email: info@kidneyurology.org

Internet: http://www.kidneyurology.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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