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.

Imaging and pathological features of gastric lesion of immunoglobulin G4-related disease: A case report and review of the recent literature.

We describe a 67-year-old man with immunoglobulin G4-related disease (IgG4-RD) presenting with optic neuropathy, dacryoadenitis, periaortitis, retroperitoneal fibrosis, and a gastric mass-like lesion. A mass-like lesion measuring 52 × 40 mm in the antrum of the stomach was found incidentally through whole-body screening for other organ involvement of IgG4-RD using contrast-enhanced computed tomography (CT). Histology of the stomach revealed that the lesion was also IgG4-related and was located in the submucosal layer extending to the subserosal region. This case suggests that the stomach can also be a site of involvement of IgG4-RD.

A case developing minimal change disease during the course of IgG4-related disease.

We describe a 66-year-old male with immunoglobulin G4-related disease (IgG4-RD) presenting with minimal change disease (MCD). Three years prior to this admission, the patient had been diagnosed with IgG4-RD. The development of sudden massive proteinuria (4+; 16.7 g/gCr) with a weight gain of 8 kg within a two-week period was noted, and nephrotic syndrome was suspected. The patient's serum IgG4 level did not increase and hypocomplementemia was not found. A renal biopsy showed no cellular infiltration in the renal interstitium, and no spiking or bubbling was found on periodic acid methenamine silver staining. On electron microscopy, foot process effacement was seen, but no subepithelial electron-dense deposits were found. The patient was diagnosed with MCD. Ten days after starting prednisolone (60 mg/day), proteinuria was negative. Since IgG4-RD and MCD share a T-helper 2-dominant immunoreaction, the development of MCD in IgG4-RD patients may reflect more than a mere coincidence.