Abstract
The estimated 20-year renal survival rate of immunoglobulin A (IgA) nephropathy is approx. 60%, but it is difficult to determine the 'big picture' for IgA nephropathy because a biopsy is essential for its diagnosis. Here we attempted to determine the longer and more precise renal prognosis of IgA nephropathy. We examined 310 patients with primary IgA nephropathy. Using the patients' clinical records and histological reports from our hospital and other clinics, we surveyed their renal prognoses and treatments within 1 year post-biopsy, and we sent questionnaires to the patients who had stopped visiting any hospital. We set renal death as the primary endpoint and analyzed factors related to renal death. The total patient cohort was 267: 159 males, 108 females; average age at biopsy, 37.7 years; average estimated glomerular filtration rate (eGFR), 69.7 mL/min/1.73m2; urinary protein, 1.3 g/day. The mean follow-up duration was prolonged to 13.8±8.9 years (vs. 9.2±8.5 years using only medical records). The 10- and 20-year follow-up rates were 61.7% and 27.3%. The 10-, 20-year renal survival rates were 83.6% and 72.5%. Lower eGFR, hypertension, and smoking were revealed as factors independently related to renal death. To study survival of relatively benign diseases such as IgA nephropathy, longer survival rate was affected by many censoring cases. The results regarding the long-term renal prognoses of IgA nephropathy patients (including those with a mild phenotype) obtained by our analysis of a questionnaire sent to the patients provided more precise and longer-term prognoses compared to earlier studies.
Highlights
In 1968 in France, Berger discovered and defined that immunoglobulin A (IgA) nephropathy is a proliferation of mesangial cells with IgA deposition [1]
Of the total of 1,277 renal pathological records, 310 patients had been diagnosed with IgA nephropathy, giving a prevalence rate of IgA nephropathy of 24.3% among all renal biopsies
We reviewed the medical records of patients with IgA nephropathy at our hospital and other hospitals and clinics, and we sent questionnaires to censored patients to determine the Mesangial hypercellularity: M0/M1 Endocapillary hypercellularity: E0/E1 Segmental glomerulosclerosis: S0/S1 Crescents: C0/C1+C2 values [number(%)] 113/88 (56.2/43.8%) 181/20 (90.0/10.0%) 142/59 (70.6%/29.4%) 98/103 (48.8/51.2%)
Summary
In 1968 in France, Berger discovered and defined that immunoglobulin A (IgA) nephropathy is a proliferation of mesangial cells with IgA deposition [1]. After Koyama’s report about these long-term outcomes, there have been almost no similar follow-up studies worldwide. For a determination of long-term outcomes in chronic diseases including IgA nephropathy, the difficulty in establishing through follow-up monitoring over a sufficiently long-enough period must be considered. Most patients with IgA nephropathy are diagnosed at a young age, and it is difficult to perform long-term follow-up on such patients because of their proclivity to change residences. Most of the previous studies surveyed only medical records or a registry, and patients who dropped out of the studies could not be followed up. We obtained each patient’s records from the referring hospital or clinic and sent a questionnaire to dropout patients to determine the follow-up condition and the recent situation of patients with IgA nephropathy
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