Clinicopathological and long-term prognostic features of membranous nephropathy with crescents: a Japanese single-center experience

Saito, Masaya; Komatsuda, Atsushi; Sato, Ryuta; Saito, Ayano; Kaga, Hajime; Abe, Fumito; Sawamura, Masato; Nara, Mizuho; Togashi, Masaru; Okuyama, Shin; Wakui, Hideki; Takahashi, Naoto

doi:10.1007/s10157-017-1465-y

Cited by 6 publications

(4 citation statements)

References 25 publications

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“…We thus propose the hypothesis that a cell-mediated inflammatory response may be involved in the transformation of crescentic MN. Tubular atrophy and interstitial fibrosis were also found to be more prominent in the crescentic MN than in the control group, which is consistent with previous studies [ 3 , 18 ]. Considering the small sample size, further studies with an expanded sample size are still needed in follow-up.…”

Section: Discussionsupporting

confidence: 92%

Clinicopathological features and prognosis of primary membranous nephropathy in combination with crescent

et al. 2023

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Objective The incidence of primary membranous nephropathy with crescentic bodies is low, but the specificity of its clinical presentation, pathology and prognosis is of great interest. In this study, we retrospectively analyzed the clinicopathological and prognostic characteristics of patients with crescentic MN in our hospital over the past 4 years. Methods Ten patients with combined crescentic primary membranous nephropathy diagnosed by renal biopsy at our hospital from 2018 to 2021 were retrospectively analysed and compared with 39 patients with PMN (simple random sample) during the same period for clinicopathological and prognostic comparisons. Results The 10 patients had higher 24 h urine protein quantification, creatinine levels on renal biopsy, interstitial fibrosis and tubular atrophy, and interstitial inflammatory cell infiltration than the control group (P < 0.05); there were no significant differences in anti-PLA2R antibodies and PLA2R staining of renal tissue (P > 0.05); At follow-up, the poor outcome of crescentic MN treatment and the low clinical remission rate were found, with the percentage of crescentic bodies being a factor in patient prognosis (P < 0.05). Conclusion Crescentic MN has a low prevalence and maybe a specific type of PMN; it has more severe clinical symptoms and pathology than PMN, and the crescentic proportion is strongly associated with renal prognosis. Intensive treatment is recommended for these patients.

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Section: Discussionsupporting

confidence: 92%

Clinicopathological features and prognosis of primary membranous nephropathy in combination with crescent

et al. 2023

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“…Mesangial proliferation is unusual and may act as an indicator of secondary forms of disease, and crescent formation and endothelial proliferation are extremely rare. Besides, tubulointerstitial lesions are widely accepted as the most important findings for disease outcome . We therefore decided to integrate only four parameters in the assessment of renal biopsies, namely: (i) the presence of FSGS, representing glomerular damage, (ii) degree of TA, (iii) severity of IF, to assess tubulointerstitial damage and (iv) presence of VH, to estimate the vasculature implication.…”

Section: Discussionmentioning

confidence: 99%

“…Besides, tubulointerstitial lesions are widely accepted as the most important findings for disease outcome. [17][18][19] We therefore decided to integrate only four parameters in the assessment of renal biopsies, namely: (i) the presence of FSGS, representing glomerular damage, (ii) degree of TA, (iii) severity of IF, to assess tubulointerstitial damage and (iv) presence of VH, to estimate the vasculature implication. Our objective was to review the importance of ordinary histological findings into a routine pathology assessment of PMN, and to provide parameters with prognostic significance and indices to guide treatment approach.…”

Section: Discussionmentioning

confidence: 99%

Histological grading in primary membranous nephropathy is essential for clinical management and predicts outcome of patients

et al. 2019

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AimsDiagnosis of primary membranous nephropathy (PMN) is mainly based on immunofluorescence/immunohistochemistry findings. However, assessment of specific features on optical microscopy can help to estimate the severity of the disease, guide treatment and predict the response. The aim of this study was to identify, classify and grade the precise histological findings in PMN to predict renal function outcome and guide treatment.Methods and resultsHistological parameters, including focal segmental sclerosis (FSGS), tubular atrophy (TA), interstitial fibrosis (IF) and vascular hyalinosis (VH), were re‐evaluated in 752 patients with PMN. Their predictive value was estimated separately, and also in a combination score (FSTIV) graded from 0 to 4. Finally, the impact of histology was assessed in the response to immunosuppressive treatment. Mean age of patients was 53.3 (15–85) years and most presented with nephrotic syndrome. FSGS was present in 32% and VH in 51% of the patients, while TA and IF were graded as stage ≥1 in 52% and 51.4%, respectively. The follow‐up period was 122.3 (112–376) months. FSGS, TA and IF and VH were associated with impaired renal function at diagnosis (P = 0.02, P < 0.0001, P = 0.001 and P = 0.02, respectively) and at the end of follow‐up (P = 0.004, P < 0.0001, P < 0.0001 and P = 0.04, respectively). In multiple regression and binary logistic analysis, the presence of FSGS and degree of TA were the most significant parameters predicting renal function outcome, defined either by eGFR (end), FSGS (r = 0.6, P < 0.0001) and TA (r = 0.6, P < 0.0001), or by the endpoint of >50% eGFR reduction, FSGS (P = 0.001) and TA (P = 0.02). Also, patients presented with FSGS, IF, VH and/or with FSTIV > 1 could benefit from immunosuppression, regardless of clinical presentation.ConclusionsThe presence and degree of four histological indices, FSGS, VH, TA and IF, assessed separately or in combination, and FSTIV score not only predict renal function outcome after long‐term follow‐up, but can also help in the choice of appropriate treatment. Decisions concerning immunosuppressive treatment can be guided by pathology regardless of clinical findings.

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“…Phospholipase A2 receptor (PLA2R) staining is positive in kidney biopsies in about 70% of so-called primary MN, with an increasing number of endogenous antigens recognized as the target in the PLA2R-negative cases [ 2 , 3 , 4 , 5 ]. Crescents in primary MN are unusual, and suggest lupus nephritis, or other additional injury, such as antineutrophil cytoplasmic antibody (ANCA)- or anti-glomerular basement membrane (anti-GBM)-associated glomerulonephritis [ 6 , 7 ]. The coexistence of nonlupus MN with crescents has been postulated to reflect initial injury by one of these mechanisms that may then unmask cryptic epitopes and lead to the second autoantibody [ 8 ].…”

Section: Introductionmentioning

confidence: 99%

Anti-Phospholipase A2 Receptor in Nonlupus Patients with Membranous Nephropathy and Crescents

et al. 2021

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Introduction: Anti-phospholipase A2 receptor (PLA2R) is detected in approximately 70% of biopsies of “primary” membranous nephropathy (MN). Crescents in MN in non-lupus patients suggest additional injury, such as antineutrophil cytoplasmic antibody (ANCA) or anti-glomerular basement membrane (anti-GBM)-associated glomerulonephritis and is postulated to reflect injury by a mechanism that unmasks cryptic epitopes leading to the second autoantibody. Methods: We studied PLA2R staining in non-lupus patients with MN and crescents. Native renal biopsies in 16 non-lupus patients with MN and crescents were stained for PLA2R. Results: The patients included 5 women and 11 men, with mean age 61 yrs and elevated serum creatinine (mean 4.68 mg/dL). Hematuria and proteinuria (mean 4.97 g/d) were documented in 13 patients. Two patients had positive serum anti-GBM antibody. Nine of eleven patients tested for ANCA were positive, with p-ANCA (n=4), c-ANCA (n=2), or both (n=1), with two not specified. On average, 27% of glomeruli had crescents. One patient had an initial biopsy with MN, 4 years later had MN with crescent, and 7 years later had rebiopsy with persistent MN with crescents. One patient had ANCA-associated vasculitis, and 5 years later had MN and crescent. The remaining 14 patients had concurrent diagnoses of MN and crescents. PLA2R was positive in 5 cases, 3 with ANCA positivity, 2 with unknown ANCA status, and none with anti-GBM disease. The patient with initial MN preceding crescent was PLA2R positive; the patient with initial ANCA-associated vasculitis preceding MN was PLA2R negative. Conclusions: Most patients (64%) presented with concomitant MN and crescents, with rare occurrence of an initial disease process followed later by the second injury. PLA2R was positive in 31% of patients, suggesting most are secondary MN. Further study to determine the cryptic epitopes may shed light on the triggering mechanisms for these rare but unlikely coincidental glomerular injuries.

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Clinicopathological and long-term prognostic features of membranous nephropathy with crescents: a Japanese single-center experience

Abstract: This is the first Japanese study demonstrating significant differences in clinicopathological and prognostic features between the 2 groups. Most patients in the crescent group may develop a long-term decline in renal function despite immunosuppressive therapy.

Cited by 6 publications

References 25 publications

Clinicopathological features and prognosis of primary membranous nephropathy in combination with crescent

Clinicopathological features and prognosis of primary membranous nephropathy in combination with crescent

Histological grading in primary membranous nephropathy is essential for clinical management and predicts outcome of patients

Anti-Phospholipase A2 Receptor in Nonlupus Patients with Membranous Nephropathy and Crescents

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