scholarly journals Clinical and pathological features of idiopathic membranous nephropathy with focal segmental sclerosis

2019 ◽  
Author(s):  
jiatong li ◽  
Bing Chen ◽  
Caifeng Gao ◽  
Jing Huang ◽  
Yongmei Wang ◽  
...  

Abstract Background Our goal was to investigate the clinical and pathological features and prognosis of idiopathic membranous nephropathy (IMN) with focal segmental lesions. Methods In our hospital, 305 patients with nephrotic syndrome confirmed by renal biopsy as IMN were divided into a non-focal segmental lesion group (FSGS- group) and a focal segmental glomerulosclerosis (FSGS) group (FSGS+ group) and retrospectively analyzed. A total of 180 patients were followed for periods ranging from six months to two years. The general clinical and pathological data of both groups were compared, and the effects of different treatment schemes on the prognosis of both groups were observed. Results The FSGS+ group had a longer disease course, higher blood pressure levels, and higher serum creatinine and β 2 -microglobulin levels than the FSGS- group (all P < 0.05). Pathologically, the FSGS+ group had increased glomerular sclerosis, glomerular mesangial hyperplasia, acute tubular lesion and chronic tubular lesion rates (all P< 0.05). The remission rate was lower in the FSGS+ group than in the FSGS- group (64.7% vs 82.2%) and was lower in patients treated with calmodulin inhibitors than in those treated with cyclophosphamide in the FSGS+ group (P < 0.01). Survival analysis showed that the FSGS+ group had a poor prognosis (χ 2 =4.377,P=0.036). Risk factor analysis suggested that age at renal biopsy (P=0.006), 24-hour urinary protein quantity (P=0.01), chronic tubulointerstitial lesions (P=0.055), and FSGS lesions (P= 0.062) were risk factors for worsening renal condition; 24-hour urinary protein quantity was an independent risk factor for worsening renal condition. Conclusions Membranous nephropathy with FSGS is a risk factor, but not an independent risk factor, for IMN. Patients with membranous nephropathy with FSGS often present hypertension and tubule injury. The nonselective drug cyclophosphamide is preferred, and calcineurin inhibitors should be used with caution. Key words Idiopathic membranous nephropathy; focal segmental sclerosis; cyclophosphamide; calmodulin inhibitor; prognosis;

2019 ◽  
Author(s):  
jiatong li ◽  
Bing Chen ◽  
Caifeng Gao ◽  
Jing Huang ◽  
Yongmei Wang ◽  
...  

Abstract Background The goal of this study was to investigate the clinical and pathological features and prognosis of idiopathic membranous nephropathy (IMN) with focal segmental lesions. Methods In our hospital, 305 patients with nephrotic syndrome confirmed as IMN by renal biopsy were divided into a non-focal segmental lesion group (FSGS- group) and a focal segmental glomerulosclerosis (FSGS) group (FSGS+ group) and retrospectively analyzed. In all, 180 patients were followed for periods ranging from six months to two years. The general clinicopathological data of both groups were compared, and the effects of different treatment schemes on the prognosis of both groups were observed. Results The FSGS+ group had a longer disease course, higher blood pressure levels, and higher serum creatinine and β2-microglobulin levels than did the FSGS- group (all P < 0.05). Pathologically, the FSGS+ group had increased glomerular sclerosis, glomerular mesangial hyperplasia, and acute and chronic tubular lesion rates (all P< 0.05). The remission rate was lower in the FSGS+ group than in the FSGS- group (64.7% vs 82.2%) and, among patients in the FSGS+ group, was lower in patients treated with calmodulin inhibitors than in those treated with cyclophosphamide (P < 0.01). Survival analysis showed that the FSGS+ group had a poor prognosis (χ2=4.377, P=0.036), and risk factor analysis suggested that age at renal biopsy (P=0.006), 24-hour urinary protein quantity (P=0.01), chronic tubulointerstitial lesions (P=0.055), and FSGS lesions (P= 0.062) were risk factors for worsening renal condition; furthermore, 24-hour urinary protein quantity was an independent risk factor for worsening renal condition. Conclusions Membranous nephropathy with FSGS is a risk factor, but not an independent risk factor, for IMN. Patients with membranous nephropathy with FSGS often present hypertension and tubule injury. The nonselective drug cyclophosphamide is preferred, and calcineurin inhibitors should be used with caution. Key words Idiopathic membranous nephropathy; focal segmental sclerosis; cyclophosphamide; calmodulin inhibitor; prognosis;


2019 ◽  
Author(s):  
jiatong li ◽  
Bing Chen ◽  
Caifeng Gao ◽  
Jing Huang ◽  
Yongmei Wang ◽  
...  

Abstract Abstract Background To investigate clinical and pathological features and prognosis of idiopathic membranous nephropathy (IMN) with focal segmental lesions. Methods In our hospital, 305 patients with biopsy-proven IMN were divided into a non-focal segmental lesion group (FSGS- group) and focal segmental glomerulosclerosis (FSGS) group (FSGS+ group) and retrospectively analyzed. The general clinical and pathological data of both groups were compared, and the effects of different treatment schemes on the prognosis of both groups were observed. Results The FSGS+ group had longer disease course; higher blood pressure levels; and higher serum creatinine andβ2-microglobulin levels than the FSGS- group (all P < 0.05). Pathologically, the FSGS+ group had increased glomerular sclerosis, glomerular mesangial hyperplasia, acute tubular lesion and chronic tubular lesion rates (all P< 0.05). The remission rate was lower in the FSGS+ group than in the FSGS- group(64.7% vs 82.2%) and was lower in association with calmodulin inhibitors than with cyclophosphamide in the FSGS+ group (P < 0.01). Survival analysis showed that the FSGS+ group had a poor prognosis(χ2=4.377,P=0.036). Risk factor analysis suggested that age at renal biopsy (P=0.006), 24-hour urinary protein quantity (P=0.01), chronic tubulointerstitial lesions (P=0.055), and FSGS lesions (P= 0.062) were risk factors for renal death; 24-hour urinary protein quantity was an independent risk factor for renal death. Conclusions Membranous nephropathy with FSGS is a risk factor for IMN but not an independent risk factor. Patients with membranous nephropathy with FSGS often present hypertension and tubule injury. Nonselective cyclophosphamide is preferred, and calcineurin inhibitors should be used with caution. Key words Idiopathic membranous nephropathy; focal segmental sclerosis; cyclophosphamide; calmodulin inhibitor; prognosis;


2019 ◽  
Vol 20 (1) ◽  
Author(s):  
Jiatong Li ◽  
Bing Chen ◽  
Caifeng Gao ◽  
Jing Huang ◽  
Yongmei Wang ◽  
...  

Abstract Background The goal of this study was to investigate the clinical and pathological features and prognosis of idiopathic membranous nephropathy (IMN) with focal segmental lesions. Methods In our hospital, 305 patients with nephrotic syndrome confirmed as IMN by renal biopsy were divided into a non-focal segmental lesion group (FSGS- group) and a focal segmental glomerulosclerosis (FSGS) group (FSGS+ group) and retrospectively analyzed. In all, 180 patients were followed for periods ranging from 6 months to 2 years. The general clinicopathological data of both groups were compared, and the effects of different treatment schemes on the prognosis of both groups were observed. Results The FSGS+ group had a longer disease course, higher blood pressure levels, and higher serum creatinine and β2-microglobulin levels than did the FSGS- group (all P < 0.05). Pathologically, the FSGS+ group had increased glomerular sclerosis, glomerular mesangial hyperplasia, and acute and chronic tubular lesion rates (all P < 0.05). The remission rate was lower in the FSGS+ group than in the FSGS- group (64.7% vs 82.2%) and, among patients in the FSGS+ group, was lower in patients treated with calmodulin inhibitors than in those treated with cyclophosphamide (P < 0.01). Survival analysis showed that the FSGS+ group had a poor prognosis (χ2 = 4.377, P = 0.036), and risk factor analysis suggested that age at renal biopsy (P = 0.006), 24-h urinary protein quantity (P = 0.01), chronic tubulointerstitial lesions (P = 0.055), and FSGS lesions (P = 0.062) were risk factors for worsening renal condition; furthermore, 24-h urinary protein quantity was an independent risk factor for worsening renal condition. Conclusions Membranous nephropathy with FSGS is a risk factor, but not an independent risk factor, for IMN. Patients with membranous nephropathy with FSGS often present hypertension and tubule injury. The nonselective drug cyclophosphamide is preferred, and calcineurin inhibitors should be used with caution.


2020 ◽  
Vol 13 (3) ◽  
pp. 152-158
Author(s):  
Lauren Copeland ◽  
Keith Gillis

Measurement of urinary protein is an essential part of the evaluation of chronic kidney disease; it has both diagnostic and prognostic significance. Proteinuria is an independent risk factor for progression of renal disease, but is also independently associated with increased cardiovascular mortality. Despite its far-reaching implications, the definition, diagnosis and treatment of proteinuria can cause confusion in primary care. Early detection of proteinuria in the context of diabetes or otherwise is vital given the potential for intervention to reduce urinary protein losses and improve renal and cardiovascular outcomes. This article will focus on the definition, potential causes and management of proteinuria, including which individuals should be referred to secondary care.


2019 ◽  
Vol 12 (1) ◽  
pp. 27-32
Author(s):  
Yosuke Inaguma ◽  
Atsutoshi Shiratori ◽  
Taku Nakagawa ◽  
Kyoko Kanda ◽  
Makiko Yoshida ◽  
...  

Background: Membranous Nephropathy (MN) is a common cause of nephrotic syndrome in adults that can also occur in children, albeit less frequently. Recently, the M-type phospholipase A2 receptor (PLA2R) was identified as the target antigen in idiopathic membranous nephropathy (IMN), making it a useful marker for diagnosis. However, there are few studies describing the potential role of PLA2R in children with IMN. The aim of this study was to clarify the involvement of PLA2R in childhood IMN. Methods: We enrolled 11 patients diagnosed with IMN from January 1998 to March 2017. We performed PLA2R staining in paraffin-embedded renal biopsy sections. The clinical data were collected from the patients’ medical records. Results: The median age at biopsy was 6 years (range, 4 to 14 years). A single 6-year-old boy among all pediatric patients with IMN had granular PLA2R staining along his glomerular capillary loops and the prevalence of PLA2R-positivity was 9%. He also showed IgG4 co-dominant staining in terms of IgG subclass. There were no apparent differences in his clinical features such as clinical data at the time of renal biopsy, the time from the treatment initiation to remission, and relapse or renal dysfunction during the follow-up period. Conclusion: We suggest that PLA2R staining can be a diagnostic tool for patients with IMN of any age, though pediatric patients with IMN have lower prevalence of PLA2R-positive staining than adult patients.


2018 ◽  
Vol 8 (2) ◽  
pp. 12-12
Author(s):  
Fatemeh Hayati ◽  
Heshmatollah Shahbazian ◽  
Ali Ghorbani ◽  
Shahla Ahmadi Halili ◽  
Eisa Rezaei ◽  
...  

Introduction: The current treatment regimens for patients with idiopathic membranous nephropathy (MN) are based on cyclophosphamide-glucocorticoid or calcineurin inhibitor-glucocorticoid. Objectives: We evaluated whether mycophenolate mofetil (MMF) -glucocorticoid could be an option for first-line therapy among these patients. Patients and Methods: In a double-blinded, randomized and controlled clinical trial, we compared the effect of MMF with cyclophosphamide in inducing complete or partial remission (PR) among patients with nephrotic syndrome due to idiopathic MN. All of the patients in both groups also received steroid, renin-angiotensin blockers and statins. Diuretics were also used in the patients who had edema. The primary end point of our study was change in urinary protein/creatinine ratio. Results: A total of 30 patients completed the study. Around 17 patients received MMF (2 g/d) and 13 patients received intravenous or oral cyclophosphamide for 6 months. At the start of the study, no significant differences in demographic and biochemical parameters of patients including the urinary protein excretion rate between two groups (P = 0.432). The proportion of proteinuria was 5235 ± 1655 mg/24 in MMF group and 8781 ± 8741 mg/24 in the cyclophosphamide group at the beginning of the study. The rate of complete and PR were 5.9% and 52.9 in MMF group versus 16.7% and 100% in cyclophosphamide group which it is significantly lower in MMF group. Kidney function was stable in both groups during treatment. Conclusions: According to the result of our study, a 6-month therapy with MMF-glucocorticoid is not recommended for treatment of patients with nephrotic syndrome due to idiopathic MN.


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