A significantly large study in Japan of over 1000 patients followed over a long time frame has shown unequivocally that IgAN is not a benign disease and leads to life threatening conditions in 50% of patients within 30 years of diagnosis. It also shows that tonsillectomy dramatically improved survival rates.
The practice of too many in medicine to consider IgAN such a long slow disease process that results in delaying treatment upon diagnosis is shown to be the wrong medical path to follow.
Here’s a summary of the paper, the full paper is available and free on line.
Prognosis in IgA Nephropathy: 30-Year Analysis of 1,012 Patients at a Single Center in Japan
Of the 1,012 patients, 40.5% were male. Mean patient age was 33±12 years and mean blood pressure was 122±17/75±13 mmHg. Mean serum creatinine concentration was 0.89±0.42 mg/dL, and mean estimated glomerular filtration rate (eGFR) was 78.5±26.2 ml/min/1.73 m2. Mean proteinuria was 1.19±1.61 g/day, and mean urinary red blood cells were 36.6±35.3/high-powered field.
Histologically, mesangial hypercellularity was present in 47.6% of patients, endothelial hypercellularity in 44.3%, segmental sclerosis in 74.6%, and tubular atrophy/interstitial fibrosis in 28.8% by Oxford classification. Initial treatment consisted of corticosteroids in 26.9% of patients, renin-angiotensin-aldosterone system inhibitor in 28.9%, and tonsillectomy plus steroids in 11.7%. The 10-, 20-, and 30-year renal survival rates were 84.3, 66.6, and 50.3%, respectively.
Tonsillectomy plus steroids dramatically improved renal outcome. Cox multivariate regression analysis showed that higher proteinuria, lower eGFR, and higher uric acid at the time of renal biopsy were independent risk factors for the development of end stage renal disease (ESRD).
IgAN is not a benign disease, with about 50% of patients progressing to ESRD within 30 years despite treatment.