From the Guidelines
IgA nephropathy typically presents with recurrent episodes of gross hematuria, often coinciding with upper respiratory tract infections, and may also be characterized by microscopic hematuria and proteinuria 1. Patients may be asymptomatic, or they may exhibit symptoms such as:
- flank pain
- hypertension
- edema In some cases, treatment with angiotensin-converting enzyme inhibitors (ACEIs) such as lisinopril, 10-20 mg daily, or angiotensin receptor blockers (ARBs) like losartan, 50-100 mg daily, may be initiated to reduce proteinuria and slow disease progression, with treatment duration individualized based on patient response and disease severity 1.
Clinical Presentations
The clinical presentations of IgA nephropathy can vary, but common features include:
- hematuria: gross or microscopic
- proteinuria: varying degrees, with higher levels indicating a worse prognosis
- hypertension: may be present, especially in patients with more severe disease
- edema: may occur, especially in patients with significant proteinuria
- flank pain: may be present, especially in patients with gross hematuria It is essential to note that the MEST-C score (mesangial and endocapillary hypercellularity, segmental sclerosis, interstitial fibrosis/tubular atrophy, and crescents) can help predict disease prognosis, but it is not currently used to determine treatment decisions 1.
Treatment Considerations
Treatment of IgA nephropathy typically involves supportive care, including RAS blockade with ACEIs or ARBs, blood pressure control, and lifestyle modifications such as dietary counseling and exercise 1. In patients with high-risk disease, immunosuppressive therapy may be considered, but the benefits and risks of such treatment must be carefully weighed, and patients should be offered the opportunity to participate in clinical trials whenever possible 1.
From the Research
Clinical Presentations of IgA Nephropathy
The clinical presentations of Immunoglobulin A (IgA) nephropathy can vary, but common manifestations include:
- Recurrent episodes of gross hematuria, often accompanied by upper respiratory tract infections 2, 3, 4
- Asymptomatic microscopic hematuria with or without proteinuria 2, 3, 4
- Persistent microscopic hematuria and proteinuria, which can progress to end-stage renal disease 3, 4, 5
- Nephrotic syndrome associated with minimal glomerular lesions, which may benefit from corticosteroid therapy 4
Disease Progression and Prognosis
The progression of IgA nephropathy can be influenced by various factors, including:
- Level of hematuria, which is independently associated with kidney disease progression 5
- Remission of hematuria, which is associated with improved kidney outcomes, particularly in patients with persistent proteinuria 5
- Presence of proteinuria, which can affect the prognosis and treatment of the disease 2, 5
- Family history, as familial IgA nephropathy may have a poorer outcome than sporadic IgA nephropathy 3
Pathogenesis and Immune Complexes
The pathogenesis of IgA nephropathy involves the deposition of immune complexes containing abnormally O-glycosylated IgA1 in the glomeruli, which can lead to glomerular injury and disease progression 6. The formation of these immune complexes is influenced by the dysregulated expression and activity of glycosyltransferases, resulting in galactose-deficient IgA1 glycoforms 6.