Takahiro Mochizuki

Clinical outcome and prognosis of anti-neutrophil cytoplasmic antibody-associated vasculitis in Japan.

Background/Aims: We conducted a broad survey of 99 patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and investigated both prognosis and outcomes. Methods: Clinical data evaluated were age, sex, patient survival, renal survival, serum albumin, serum creatinine, urinary protein, hematuria, C-reactive protein (CRP), ANCA titer, IgG and the Birmingham Vasculitis Activity Score (BVAS). Results: The patient survival rate at 6 months after onset was 84.8%, and that at 2 years after onset was 82.0%. Most deaths were within 6 months of onset. Infection accounted for 9 deaths (60.0%). Infection together with pulmonary involvement of active vasculitis accounted for 2 deaths (13.3%). Organ-specific involvement of active vasculitis alone caused 3 deaths (20.0%). Others died of cardiac events. At 1 and 3 months after onset, BVAS (p < 0.0001, p = 0.002), albumin (p = 0.006, p = 0.0004) and CRP (p = 0.04, p = 0.0002) were also associated with patient death. Conclusion: To improve the prognosis of those with ANCA-associated vasculitis, the intensity of initial treatment should be aimed at disease severity. Employing BVAS improved the ability to evaluate therapeutic responses. Finally, prescription with sulfamethoxazole-trimethoprim during the induction therapy with immunosuppressive agents may be advised.

Maximal glomerular diameter as a 10-year prognostic indicator for IgA nephropathy

BACKGROUND Although there have been many reports on clinicopathological studies of immunoglobulin A nephropathy (IgAN), reliable outcome predictors are still lacking. We therefore assessed maximal glomerular diameter (Max GD), an indicator of glomerular size, as a predictor of the long-term evolution of renal histopathology. METHODS Forty-three adult patients, diagnosed with IgAN, who had estimated glomerular filtration rate (eGFR) ≥50 mL/min/1.73 m(2), were enrolled in this study. Prognostic variables for renal survival were examined by using the multivariate Cox proportional hazards method. The optimal cut-off value of Max GD was 242.3 μm (AUC = 0.78, sensitivity = 62.5%, specificity = 81.5%) by using receiver operating characteristics analysis. In order to assess the characteristics of glomerular hypertrophy, we divided the cases into two groups according to the Max GD value (Group A, ≥242 μm; Group B, <242 μm). Renal survival was also assessed by Kaplan-Meier curves with the log-rank test. RESULTS The Max GD was significantly correlated with age, body mass index and serum triglyceride levels at the time of renal biopsy. During the 10-year follow-up period, the Max GD was significantly correlated with eGFR decline per year, and proteinuria, but not with hematuria. A multiple regression analysis by the Cox method adjusted for age, sex and eGFR showed that the Max GD values were significantly associated with a 1.5-fold increase in serum creatinine (Cr) values (hazard ratio = 1.04, P = 0.03). Renal function in 66.7% of the patients whose Max GD was ≥242 μm had at least a 1.5-fold increase in their serum Cr value at the 10-year follow-up examination (log-rank, P = 0.003). CONCLUSIONS The results of this study suggest that Max GD is a simple quantitative prognostic indicator of the disease progression in IgAN patients.

Serum Hepcidin Levels and Reticulocyte Hemoglobin Concentrations as Indicators of the Iron Status of Peritoneal Dialysis Patients

Hepcidin is the key mediator of renal anemia, and reliable measurement of serum hepcidin levels has been made possible by the ProteinChip system. We therefore investigated the iron status and serum hepcidin levels of peritoneal dialysis (PD) patients who had not received frequent doses of an erythrocytosis-stimulating agent (ESA) and had not received iron therapy. In addition to the usual iron parameters, the iron status of erythrocytes can be determined by measuring reticulocyte hemoglobin (RET-He). The mean serum hepcidin level of the PD patients (n = 52) was 80.7 ng/mL. Their serum hepcidin levels were significantly positively correlated with their serum ferritin levels and transferrin saturation (TSAT) levels, but no correlations were found between their serum hepcidin levels and RET-He levels, thereby suggesting that hepcidin has no effect on the iron dynamics of reticulocytes. Since low serum levels of CRP and IL-6, biomarkers of inflammation, were not correlated with the serum hepcidin levels, there is likely to be a threshold for induction of hepcidin expression by inflammation.