Management of IgAN

CE / CME

Improving Outcomes in IgAN: Expert Guidance on Evolving Management and Treatment

Physician Assistants/Physician Associates: 1.00 AAPA Category 1 CME credit

Nurses: 1.00 Nursing contact hour

Physicians: maximum of 1.00 AMA PRA Category 1 Credit

Released: July 01, 2024

Expiration: June 30, 2025

Activity

Progress
1 2
Course Completed

Epidemiology and Diagnosis

IgAN is an important disease, and I hope to help your understanding of the disease in this module and to illustrate how the pathogenesis is stimulating discovery into new therapies to ameliorate outcomes. IgAN is the most common form of glomerulonephritis worldwide. There are some studies that have indicated that subclinical IgAN may be detectable in autopsy studies in up to 16% of people in some high-prevalence areas.

The diagnosed incidence in the United States is approximately 1 of 100,000/year, but incidence is closely related to prevalence because the disease is, once diagnosed, a chronic disease; for most patients, it will stay with them for life. We see geographic differences in the epidemiology with more common or more prevalent disease in East Asia and Northern Europe compared with much lower prevalence in sub-Saharan Africa.

The disease requires a kidney biopsy to make a definitive diagnosis. The sine qua non of the diagnosis is dominant or codominant mesangial deposits of IgA in the kidney.1-6

Listen to this brief audio clip to hear a patient’s experience with her IgAN diagnosis.  

Clinical Manifestations

There are various ways that IgAN manifests clinically. Often, despite the patient being asymptomatic, it is discovered when microscopic hematuria with variable amounts of proteinuria is detected via routine urine tests. It may also present with gross hematuria in the setting of mucosal infections. We call this synpharyngitic hematuria because it very often coincides with pharyngeal infections. IgAN is characterized not only by microhematuria, but also by proteinuria. As time passes, proteinuria can increase and be associated with a progression of chronic kidney disease (CKD) and loss of glomerular filtration rate (GFR).

There are other ways in which it can present besides the more typical sort of chronic glomerulonephritis form. There are rapidly progressive forms that can present as a crescentic glomerulonephritis that causes rapid kidney failure over weeks or months. There is a form of acute kidney injury that happens in the setting of severe gross hematuria, that causes tubular injury from the blood in the tubules. And some patients with IgAN will actually present with nephrotic syndrome.

There is also a systemic relative of IgAN called IgA vasculitis, which shares many of the same pathogenetic and pathogenic mechanisms, but where the IgA is deposited in small vessels across the body, not just in the kidney. IgA vasculitis tends to affect young people and is also known as Henoch-Schonlein purpura. When it resolves, a person may be left with renal changes that look indistinguishable from IgAN.8

Listen to this brief audio clip to hear about the patient burden related to an IgAN diagnosis.

Burden of IgA Nephropathy

IgAN is a morbid disease. It can affect people at any age but is particularly common in people who are young and middle aged and is often diagnosed in patients in their teens to 30s. It affects people in particular in the prime of their life, including key years for economic productivity and building a family. In fact, because of its early diagnosis, some studies suggest that in the majority of patients, it will lead to progressive kidney disease and a high lifetime likelihood of progression to end-stage kidney disease. Therefore, patients with this diagnosis may bring with it a fear of progression to end-stage kidney disease and the knowledge of the high likelihood of needing dialysis or a kidney transplant within their productive lifetime. That has implications, of course, for the patients and their approach to life. It also has socioeconomic impact as far as productivity and quality of life, which can be attributed also to some of the treatments for the disease.9-12

IgA Nephropathy Care Costs and Utilization

Here is an example from a study showing the care cost and utilization in patients with IgAN. The costs of care go up as CKD stage increases: the more severe the CKD, the lower the GFR, the higher the cost of care. These costs are substantial, and they last for a patient's lifetime in many cases.13

Listen to this brief audio clip to hear about the impact of IgAN on a patient’s emotional and mental well-being.