Long-term outcomes of lupus nephritis with low-level proteinuria: a multicenter, retrospective study.

OBJECTIVES: Reportedly, patients with lupus nephritis (LN) and low-level proteinuria have favorable short-term renal outcomes. We aimed to clarify the long-term renal outcomes and overall survival of them, and the significance of renal biopsy in the early phase with low-level proteinuria. METHODS: We included 144 Japanese patients with biopsy-proven LN from ten hospitals. Low-level proteinuria was defined by a urine protein: creatinine ratio (UPCR) of ≤ 1 g/gCr based on previous reports. The outcomes were end-stage renal disease (ESRD) and death. RESULTS: Compared with patients with high-level proteinuria (UPCR > 1), those with low-level proteinuria (n = 67 [46.5%]) had significantly improved renal function at the time of renal biopsy, and low activity index and chronicity index (CI) while the frequency of class III/IV was similar (79.1% vs 84.4%, p = 0.409). In patients with low-level proteinuria, cyclophosphamide usage was less, and the incidence of ESRD (3.0% vs 13.0%, p = 0.036) or death (3.0% vs 16.9%, p = 0.006) during the total observation period (median, 72 months) were low. Kaplan-Meier analysis showed significant differences in the incidence of ESRD and death between the groups. Multivariate Cox regression analysis revealed that the significant risk factors for ESRD were high CI and hypertension, whereas those for death were increased age and high-level proteinuria. CONCLUSION: Patients with LN and low-level proteinuria had favorable long-term renal and life outcomes. As these patients have substantial active pathological lesions, renal biopsy in the early phase with low-level proteinuria could enable early diagnosis and treatment and thus improve prognosis.

Clues to mortality trends and their related factors in IgG4-related disease: A Japanese single-centre retrospective study.

OBJECTIVES: This study aimed to clarify mortality trends and their related factors in immunoglobulin G4-related disease (IgG4-RD) with various organ involvement. METHODS: We retrospectively reviewed the medical records of patients with IgG4-RD at a single rheumatology centre in Japan. We calculated the standardized mortality ratio using Japanese national mortality statistics. Cox regression analyses were also performed to assess mortality-related factors. RESULTS: A total of 179 patients with IgG4-RD were included with a median follow-up period of 47 months. The standardized mortality ratio in our cohort was 0.86 (95% confidence interval 0.41-1.59). Univariate Cox regression analyses indicated that the number of affected organs at diagnosis (hazard ratio 1.45, 95% confidence interval 1.02-2.05), estimated glomerular infiltration rate <45 ml/min/1.73 m2 at diagnosis (vs. ≥45, hazard ratio 8.48, 95% confidence interval 2.42-29.79), and the presence of malignancy during the clinical course (hazard ratio 5.85, 95% confidence interval 1.62-21.15) had a significant impact on the time to death. CONCLUSIONS: Our findings suggest that in the rheumatology department, IgG4-RD does not significantly affect long-term patient survival. However, multi-organ involvement, renal dysfunction, and malignancy may be associated with higher mortality trends in IgG4-RD. Early detection and appropriate management of risk factors may improve the long-term prognosis of patients with IgG4-RD.

Pneumonia and central nervous system infection caused by reactivation of varicella-zoster virus in a living-donor kidney transplantation patient: case report and review of the literature.

Varicella-zoster virus (VZV) typically causes herpes zoster in the elderly due to reactivation, but immunocompromised individuals may develop organ damage such as pneumonia with a poor prognosis. We herein report a case of pneumonia and central nervous system (CNS) infection caused by reactivation of VZV in a 50-year-old man who had received a living-donor kidney transplant. We also conducted a literature review of adult cases with pneumonia or CNS infection caused by VZV after kidney transplantation. It showed that there are cases in which eruptions appeared upto 21 days after the onset of the disease and others in which eruptions did not appear at any time during the clinical course. Furthermore, there may be a wide variety of intervals from kidney transplantation to VZV infection (including both primary infection and reactivation of VZV), ranging from 2 weeks to 11 years. Therefore, it should be kept in mind that kidney transplant patients are always at high risk of VZV infection, as early recognition and treatment of the disease improves its prognosis. Although the diagnosis of varicella pneumonia is generally made by PCR test of bronchoalveolar lavage fluid, our case experience suggests that the less invasive PCR test of sputum may be useful for rapid and accurate diagnosis. The efficacy of inactivated recombinant zoster vaccine in immunocompromised individuals at high risk of reactivation of VZV also needs to be examined in the future.