OBJECTIVES: This literature review aimed to gather and narratively synthesize evidence on the clinical, economic and humanistic burden associated with IgAN. METHODS: EMBASE and Medline databases were searched for relevant English-language publications (2000–2017), which were selected based on pre-defined inclusion criteria through a two-step screening process; (i) abstract and (ii) full-text screening. RESULTS: The review identified 52 heterogeneous publications that varied with respect to the number of IgAN patients included (19–11,963); study settings (single-center [n=30] vs. multi-center [n=15] vs. not reported [n=7]) and geographies (Asia [n=32], Europe [n=11], North America [n=4] and not reported [n=5]). While all publications provided evidence on clinical burden, none had evidence on economic or humanistic burden. At diagnosis, the mean age ranged from 25.2–50.3 years, and the mean proteinuria ranged from 0.75–3.04 g/day. Hypertension was the most common comorbid condition. The publications, though heterogeneous, provided information on the disease progression at different time-points of follow-up. Within five years of follow-up, a ≥50% decline in glomerular filtration rate (GFR) was observed in 0.6% (Swedish patients) to 26.9% (Pacific Asian patients). The proportion of patients progressing to end-stage renal disease (ESRD) ranged from 4.0%–11.2% at 5 years; 13.4–22% at 6–10 years and 31.9% at 15 years of follow-up. Histological presentations of tubular atrophy, interstitial fibrosis and high histological disease grade (III/IV) were associated with progression to ESRD (P<0.01 each), along with advanced age, hypertension and Pacific Asian population (P<0.001 each). IgAN patients who underwent renal transplantation was as high as 27.1% at ~5 years of follow-up. CONCLUSIONS: The high clinical burden associated with IgAN confirms that there is an unmet need for interventions that can delay the progression of this rare indication. Additionally, there is a necessity to undertake research on the humanistic and economic burden associated with IgAN.