Prognostic Factors in Anti-Neutrophil Cytoplasmic Antibody-Associated Glomerulonephritis with Severe Glomerular Sclerosis: A National Registry-Based Cohort Study

Background. Classification of patients with anti-neutrophil cytoplasmic antibody-associated glomerulonephritis (ANCA-GN) into histological classes is useful for predicting a patient’s risk of progression to end-stage renal disease (ESRD). However, even in the worst prognostic group, the 5-year end-stage renal disease-free survival rate is as high as 50%. Objectives. To investigate those prognostic factors indicative of progression to ESRD in patients with ANCA-GN and sclerosing histology. Methods. Patients from the Norwegian Kidney Biopsy Registry between 1991 and 2012 who had biopsy verified pauci-immune glomerulonephritis, positive ANCA serology, and sclerosing histology were included. Cases with ESRD during follow-up were identified via linkage with the Norwegian Renal Registry. Potential prognostic factors with relevant cut-offs were compared in patients with and without progression to ESRD during follow-up. Results. Of 23 included patients, 10 progressed to ESRD. ESRD patients had a lower initial estimated glomerular filtration rate (eGFR; 21 versus 52 ml/min/1.73 m2) and a lower percentage of normal glomeruli (4% versus 15%). Five-year risks of ESRD with eGFR >15 versus ≤15 ml/min/1.73 m2 were 77% and 15%, with percentage normal glomeruli >10% versus ≤10%, 83% and 39%. Conclusions. eGFR and percentage of normal glomeruli are strong risk factors for ESRD in ANCA-GN with sclerosing histology.

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Impact of Proteinase 3 versus Myeloperoxidase Positivity on Risk of End-Stage Renal Disease in ANCA-Associated Glomerulonephritis Stratified by Histological Classification: A Population-Based Cohort Study

Disease Markers ◽

10.1155/2018/3251517 ◽

2018 ◽

Vol 2018 ◽

pp. 1-7 ◽

Cited By ~ 1

Author(s):

Vilde Solbakken ◽

Anne-Siri Fismen ◽

Leif Bostad ◽

Rune Bjørneklett

Keyword(s):

Renal Disease ◽

End Stage Renal Disease ◽

Antineutrophil Cytoplasmic Antibody ◽

Lower Percentage ◽

Proteinase 3 ◽

Histological Classification ◽

Free Survival ◽

Stage Renal Disease ◽

End Stage

Background. End-stage renal disease (ESRD) risk in patients with antineutrophil cytoplasmic antibody- (ANCA-) associated glomerulonephritis (ANCA-GN) according to ANCA serotype and stratified by histological classification has not been previously investigated. Methods. Patients from the Norwegian Kidney Biopsy Registry (NKBR) between 1991 and 2012 who had biopsy-verified pauci-immune glomerulonephritis and positive antineutrophil cytoplasmic antibody serology were included. Cases with ESRD during follow-up were identified in the Norwegian Renal Registry. ESRD-free survival with proteinase 3 (PR3) versus myeloperoxidase- (MPO-) ANCA positivity stratified into 4 histological classes was investigated. Results. Three hundred fifty-eight patients, of whom 87 progressed to ESRD during follow-up, were included. Patients with PR3- as compared to MPO-ANCA were younger (58 versus 64 years, p=0.001), had a higher percentage of males (62 versus 41%, p<0.001), had a lower percentage with a sclerozing glomerulonephritis pattern (4 versus 16%, p<0.001), and had a significantly higher cumulative ESRD-free survival (90 versus 80%, p=0.007) at 1-year follow-up. No significant differences in cumulative ESRD-free survival with PR3- as compared to MPO-ANCA were observed by histological stratification. Conclusion. Advanced glomerular sclerosis is found more frequently in patients with MPO-ANCA, explaining the higher risk of ESRD. ANCA serotypes have no impact on prognosis of patients with similar histological findings.

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Fibrillary glomerulonephritis with a favourable prognosis of 26 years

Journal of Nephropathology ◽

10.34172/jnp.2021.44 ◽

2020 ◽

Vol 10 (4) ◽

pp. e44-e44

Author(s):

David Micarelli ◽

Valentina Pistolesi ◽

Emanuela Cristi ◽

Anna Rita Taddei ◽

Ilaria Serriello ◽

...

Keyword(s):

Renal Function ◽

Renal Disease ◽

End Stage Renal Disease ◽

Kidney Biopsy ◽

Clinical Course ◽

Glomerular Sclerosis ◽

Fibrillary Glomerulonephritis ◽

Stage Renal Disease ◽

End Stage

Fibrillary glomerulonephritis (FGN) is a rare glomerular disease. The prognosis is usually unfavorable with nearly half of patients progressing to end-stage renal disease within 4 years. We report a case of biopsy-proven FGN characterized by an unusual benign clinical course in which a kidney biopsy, repeated after an extended follow-up of 26 years, confirmed the presence of fibrils deposition. In 1993, a 32-year-old Caucasian man was admitted to our nephrology ward because of macroscopic hematuria. Renal function was normal. Kidney biopsy displayed an FGN with mesangial pattern. The patient was treated with lisinopril, titrated for blood pressure; the therapy was maintained during 26 years of follow-up. The yearly slope of estimated glomerular filtration rate was -3.17 mL/ min). Starting from March 2018, a rapid worsening of renal function was observed and proteinuria increased up to a nephrotic range. We planned a second renal biopsy to assess the cause of the rapid change of clinical course. The diagnosis of FGN on advanced sclerosis was made, and the severity of glomerular sclerosis. We report a case of FGN with an unusually benign clinical course, characterized by a slow progression to end-stage renal disease over a very extended follow-up time; thus, to better clarify the reason for renal function worsening, a second renal biopsy was performed. The persistence of fibrils deposition confirmed the initial diagnosis of FGN, and a histological pattern characterized by global glomerular sclerosis and interstitial fibrosis has been observed.

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MO282EARLY RENAL RECOVERY AFTER A FIRST FLARE OF PAUCI IMMUNE GLOMERULONEPHRITIS

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfab104.0040 ◽

2021 ◽

Vol 36 (Supplement_1) ◽

Author(s):

Jeremy Zaworski ◽

Cyrille Vandenbussche ◽

Pierre Bataille ◽

Eric Hachulla ◽

Francois Glowacki ◽

...

Keyword(s):

Renal Disease ◽

End Stage Renal Disease ◽

Interstitial Fibrosis ◽

Univariate Analysis ◽

Renal Recovery ◽

Neurological Involvement ◽

Factors Associated ◽

Stage Renal Disease ◽

End Stage

Abstract Background and Aims Renal involvement is a severe manifestation of ANCA-associated vasculitis. Patients often progress to end-stage renal disease. The potential for renal recovery after a first flare has seldom been studied. Our objectives were to describe the evolution of the estimated glomerular filtration rate (eGFR) and identify factors associated with the change in eGFR between diagnosis and follow-up at 3 months (ΔeGFRM0–M3) in a cohort of patients with a first flare of pauci-immune glomerulonephritis. Methods This was a retrospective study over the period 2003–2018 of incident patients in the Nord-Pas-de-Calais (France). Patients were recruited if they had a first histologically-proven flare of pauci immune glomerulonephritis with at least 1 year of follow up. Kidney function was estimated with MDRD-equation and analysed at diagnosis, 3rd, 6th and 12th months. The primary outcome was ΔeGFRM0–M3. Factors evaluated were histological (Berden classification, interstitial fibrosis, percentage of crescents), clinical (extra-renal manifestations, sex, age) or biological (severity of acute kidney injury, dialysis, ANCA subtype). Results One hundred and seventy-seven patients were included. The eGFR at 3 months was significantly higher than at diagnosis (mean ± standard deviation, 40 ± 24 vs 28 ± 26 ml/min/1.73 m2, p < 0.001), with a ΔeGFRM0–M3 of 12 ± 19 ml/min/1.73 m2. The eGFR at 12 months was higher than at 3 months (44 ± 13 vs 40 ± 24 ml/min/1.73m2, p = 0.003). The factors significantly associated with ΔeGFRM0–M3 in univariate analysis were: sclerotic class according to Berden classification, percentage of interstitial fibrosis, percentage of cellular crescents, acute tubular necrosis, neurological involvement. The factors associated with ΔeGFRM0–M3 in multivariate analysis were the percentage of cellular crescents and neurological involvement. The mean increase in eGFR was 2.90 ± 0.06 ml/min/1.73m2 for every 10-point gain in the percentage of cellular crescents. ΔeGFRM0–M3 was not associated with the risks of end-stage renal disease or death in long-term follow-up. Conclusions Early renal recovery after a first flare of pauci-immune glomerulonephritis occurred mainly in the first three months of treatment. The percentage of cellular crescents was the main independent predictor of early renal recovery.

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