Disease Directory IgA Nephropathy
Immune

IgA Nephropathy

Also known as: Berger disease, IgA glomerulonephritis, mesangial IgA nephropathy

Prevalence

25 in 100,000; most common primary glomerulonephritis worldwide

Onset

Young adulthood (2nd-3rd decade), occasionally pediatric

Type

Autoimmune glomerulonephritis (IgA mediated)

About IgA Nephropathy

IgA Nephropathy is the most prevalent primary glomerulonephritis globally, caused by mesangial deposition of poorly galactosylated polymeric IgA1 and subsequent complement activation and immune complex formation, leading to glomerular inflammation and progressive kidney injury. Clinical presentation ranges from asymptomatic hematuria detected incidentally to nephrotic syndrome and rapidly progressive glomerulonephritis, with approximately 30-40% of patients reaching end-stage kidney disease within 20-30 years. The expanding pipeline of targeted therapies — including endothelin-angiotensin system inhibitors, BAFF/APRIL inhibitors, complement pathway blockers, and sparsentan — has made IgA nephropathy one of the most actively trialed rare kidney diseases.

Common Clinical Features

Macroscopic hematuria coinciding with upper respiratory infections Persistent microscopic hematuria with or without proteinuria Hypertension Proteinuria (variable, up to nephrotic range) Declining estimated glomerular filtration rate Flank pain during gross hematuria episodes Edema in nephrotic presentations

Clinical Trial Eligibility Tips

What to know before applying to IgA Nephropathy trials.

Kidney biopsy with Oxford classification (MEST-C score) is required by virtually all trials; ensure pathology report includes mesangial IgA deposit confirmation on immunofluorescence

Proteinuria threshold (commonly >1 g/day or >0.5 g/g urine protein:creatinine ratio) and eGFR range (often 30-90 mL/min/1.73m2) are the primary eligibility gatekeepers; provide 24-hour urine or spot ratio from last 3 months

Maximum-tolerated RAS blockade is a prerequisite for most trials; document ACE inhibitor or ARB dose and duration along with current blood pressure readings

Patient Resources

Patient Organization

IgA Nephropathy Foundation

Visit website ↗

Natural History Registry

IgAN Registry

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:93024)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

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Find recruiting IgA Nephropathy trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for IgA Nephropathy. Updated daily.

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