IgA nephropathy is an autoimmune kidney disorder characterized by settlement of immunoglobulin A (IgA), a protein that helps the body fight infections, in the kidneys. This leads to inflammation that makes it difficult for the kidneys to effectively filter blood, causing blood and protein in the urine. IgA nephropathy may also be called Berger disease.
Jonathan Barratt, PhD, FRCP, Professor of Renal Medicine, University of Leicester, UK, gives an overview of the disease.
Etiology
IgA nephropathy causes destruction of the glomerular basement membrane. Often, an infectious disease precedes the manifestation of the disorder, but in most instances, the cause of this condition is unknown. The disease may be familial (10% of cases) or sporadic/idiopathic (90% of cases). However, certain disorders, such as liver cirrhosis, celiac disease, and HIV infection have been linked with the disorder.
Signs and Symptoms
Early stage IgA nephropathy usually does not show any signs or symptoms of the disease. The first sign of this condition is typically blood in the urine. Other symptoms may include:
- Hematuria
- Proteinuria
- Swelling in legs, feet, ankles, or other parts of the body (edema)
The disorder may eventually cause end-stage kidney disease.
Diagnosis
IgA nephropathy is diagnosed by a urine analysis, kidney biopsy, and Immunofluorescence demonstrating the deposit of IgA in the glomerular basement membrane.
Management Strategies and Treatment
Currently, treatment options consist mostly of symptom management. These may include angiotensin-converting enzyme inhibitors or angiotensin receptor blockers, corticosteroids, and liver transplantation.
Richard Lafayette, MD, FACP, Director of the Stanford Glomerular Disease Center, Editor-in-Chief of ASN Kidney News, and Rheumatologist at Stanford Health Care, describes the current standard of care.
Andy Udell, President of Calliditas Therapeutics North America, discusses IgA nephropathy and the use of budesonide (Tarpeyo) to treat this rare disease.
The following therapies have been approved by the U.S. Food and Drug Administration (FDA):
- Budesonide (Brand name: Tarpeyo)
Clinical Trials and Studies
For a full list of clinical trials relating to IgA nephropathy, click here.
Resources
To learn more about rare autoimmune disorders, visit https://checkrare.com/diseases/autoimmune-auto-inflammatory-disorders/
References
Rawla P, Limaiem F, Hashmi MF. IgA Nephropathy (Berger Disease). [Updated 2023]. In: StatPearls [Internet]. Available at https://www.ncbi.nlm.nih.gov/books/NBK538214/
