P0476THE ABSENCE OF REMISSION PREDICTS MORTALITY IN RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Leydy Yohana Gil Giraldo ◽  
Patricia Muñoz Ramos ◽  
Antonio Carlos Fernández Perpen ◽  
Borja Quiroga
Keyword(s):  
Renal Function ◽  
Associated Factors ◽  
Cox Regression ◽  
Independent Predictor ◽  
Rapidly Progressive Glomerulonephritis ◽  
Epidemiological Data ◽  
Induction Treatment ◽  
Stage Renal Disease ◽  
End Stage

Abstract Background and Aims Rapidly progressive glomerulonephritis (RPGN) encompasses a group of diseases with a common histology in which the absence of treatment progress to end-stage renal disease. Induction treatment includes the use of immunosuppressants, and in certain cases, plasmapheresis. The final objective of this study was to determine the long-term prognosis of the RPGN. Method A retrospective observational study was conducted, including patients diagnosed with RPGN between 2004 and 2019. Baseline epidemiological data and comorbidities were collected, as well as renal function and treatment at the time of diagnosis. During the follow up [median of 42 (5-101 months)], we analyzed the evolution of renal function, mortality and associated factors. Results Forty-three patients (65% women) were included, with a mean age of 70 ± 16 years. At the time of diagnosis, mean creatinine was 4.8 ± 2.6 mg/dl, proteinuria 1094 ± 856 mg/day and 37 patients (86%) presented hematuria Thirty-one patients (72%) presented positivity for antibodies against the neutrophil cytoplasm, 7 (16%) for antibodies against the glomerular basement membrane and 5 (12%) for both. Regarding the induction treatment, 41 patients received cyclophosphamide and corticoids and two patients received rituximab. Seventeen (31%) plasmapheresis were performed with a median of 7 (6-7 sessions). At 6 months, 55% of the patients presented remission (15 patients complete remission and 8 patients partial remission). The median creatinine was 1.9 (1.2-3.1) mg/dl and the proteinuria was 380 (85 -542) mg/day (p<0.0001 compared to the initial data). At that time, 21% (9) of the patients needed dialysis. Associated factors with the absence of remission were diabetes mellitus (p= 0.016), creatinine at diagnosis (p= 0.002) and the need for hemodialysis at admission (p<0.0001). The only independent predictor of remission was initial creatinine (HR 0.5 [0.3-0.9], p= 0.048). During follow up, renal function improved with a median of creatinine at 18 months of 1.6 (1.2 – 2.9) mg/dl and 1.5 (0.8-2.4) mg/dl at the end. Twelve (28%) patients died during follow up. Associated factors with mortality were age (p=0.02), the need for hemodialysis (p=0.015) and the absence of remission at 6 months (p=0.012) (figure 1). An adjusted model using Cox regression demonstrated that the absence of remission was an independent predictor of mortality (HR 0.2 [0.5-0.8], p= 0.032). Conclusion Initial renal function and 6-month remission predicts mortality in the RPGN.

scholarly journals Vasculitis ANCA: Alternativas de tratamiento y las expectativas en los pacientes

2021 ◽  
pp. 1-2
Author(s):  
Carolina Aguilar-Martínez 
Keyword(s):  
Systematic Review ◽  
Cox Regression ◽  
Meta Analysis ◽  
Significant Risk ◽  
Limiting Factor ◽  
Significant Survival ◽  
Stage Renal Disease ◽  
End Stage ◽  
Immunosuppression Therapy

<b>Background:</b> The benefits of treating anti-neutrophil cytoplasmic autoantibody-associated vasculitis (AAV) in advancing age remains unclear with most published studies defining elderly as ≥65 years. This study aims to determine outcomes of induction immunosuppression in patients aged ≥75 years. <b>Methods:</b> A cohort of patients aged ≥75 years with a diagnosis of AAV between 2006 and 2018 was constructed from 2 centres. Follow-up was to 2 years or death. Analysis included multivariable Cox regression to compare mortality and end-stage renal disease (ESRD) based on receipt of induction immunosuppression therapy with either cyclophosphamide or rituximab. A systematic review of outcome studies was subsequently undertaken amongst this patient group through Pubmed, Cochrane and Embase databases from inception until October 16, 2019. <b>Results:</b> Sixty-seven patients were identified. Mean age was 79 ± 2.9 years and 82% (<i>n</i> = 55) received induction immunosuppression. Following systematic review, 4 studies were eligible for inclusion, yielding a combined total of 290 patients inclusive of our cohort. The aggregated 1-year mortality irrespective of treatment was 31% (95% CI 25–36%). Within our cohort, induction immunosuppression therapy was associated with a significantly lower 2-year mortality risk (hazard ratio [HR] 0.29 [95% CI 0.09–0.93]). The pooled HR by meta-analysis confirmed this with a significant risk reduction for death (HR 0.31 [95% CI 0.16–0.57], <i>I</i><sup>2</sup> = 0%). Treated patients had a lower pooled rate of ESRD, but was not statistically significant (HR 0.71 [95% CI 0.15–3.35]). <b>Conclusion:</b> This meta-analysis suggests that patients ≥75 years with AAV do benefit from induction immunosuppression with a significant survival benefit. Age alone should not be a limiting factor when considering treatment.

Crescentic glomerulonephritis

2009 ◽  
pp. 399-434
Author(s):  
Patrick Nachman ◽  
Richard J. Glassock
Keyword(s):  
Renal Function ◽  
End Stage Renal Disease ◽  
Crescentic Glomerulonephritis ◽  
Rapidly Progressive Glomerulonephritis ◽  
Clinical Syndrome ◽  
Aggressive Treatment ◽  
Stage Renal Disease ◽  
End Stage ◽  
Glomerular Tuft

The term crescentic glomerulonephritis (CrGN) refers to a diverse collection of disorders of widely different etiology and pathogenesis having in common the development of extensive proliferation of cells within Bowman's space (Couser, 1988; Glassock et al., 1995; Nachman et al., 1998; Pusey and Rees, 1998; Morgan et al., 2006; Lionaki, et al., 2007). The resulting accumulation of cells gives rise to a ‘crescent’ enveloping the glomerular tuft itself. Polymerization of fibrinogen in Bowman's space due to passage of fibrinogen through gaps in the capillary wall, the elaboration of procoagulant factors by infiltrating monocytes and impaired fibrinolysis all contribute to the pathogenesis of the crescent (Couser, 1988, Glassock et al., 1995). Usually 〉50% of glomeruli are involved with crescentic lesions. Such patients also frequently manifest rapid and progressive deterioration of renal function leading to the clinical syndrome of rapidly progressive glomerulonephritis. Early and aggressive treatment can often delay or prevent the development of end-stage renal disease (ESRD). See Table 10.1 for an etiologic and pathogenetic classification of CrGN.

scholarly journals P0181PERSISTENT C3 HYPOCOMPLEMENTEMIA AND MODERATE TUBULOINTERSTITIAL SCARRING AS EARLY PREDICTORS OF END-STAGE RENAL DISEASE IN LUPUS NEPHRITIS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Giovanni Maria Rossi ◽  
Francesco Peyronel ◽  
Marco Delsante ◽  
Avi Z Rosenberg ◽  
Paride Fenaroli ◽  
...  
Keyword(s):  
End Stage Renal Disease ◽  
Regression Models ◽  
Kidney Biopsy ◽  
Cox Regression ◽  
Activity Index ◽  
Renal Recovery ◽  
Early Predictors ◽  
Stage Renal Disease ◽  
End Stage

Abstract Background and Aims The prognosis of lupus nephritis (LN) has become progressively more favorable thanks to the introduction of cyclophosphamide and mycophenolate as the mainstay of induction of remission treatment regimens. However, 10-15% of patients still progress to end-stage renal disease (ESRD). Early predictors of ESRD, i.e. in the first six months between kidney biopsy and the completion of induction, are currently limited to few histological and clinical features: ≥ 25% interstitial fibrosis and tubular atrophy (IFTA), fibrinoid necrosis, fibrous crescents, and thrombotic microangiopathy (TMA) [Rijnink EC et al CJASN 2017; Song D Arthritis Res Ther 2013]; lack of decrease in proteinuria &lt; 0.5 g/24-h at 3 and 6 months from kidney biopsy [Tamirou F Ann Rheum Dis 2016], baseline GFR ≤ 90 ml/min/1.73 m2, lack of decrease in urinary protein-to-creatinine ratio (UPCR) &lt; 1 and anti-dsDNA positivity at the end of induction [Dall’Era M Lupus Sci Med 2015]. In this study we sought to identify further clinical and histological predictors of ESRD in LN. Methods Adult patients diagnosed with LN between 1995 and 2018 in two centers (NIAMS, Bethesda, Maryland, USA, and Nefrologia, AOU di Parma, Italy) were retrospectively identified. Patients with available serum C3 and C4 levels at the time of biopsy and 6 months thereafter, and a follow-up of at least 6 months, were included. Baseline and follow-up data (until March 2019) including age, sex, ethnicity, clinical, histological and laboratory findings were collected. Histology slides were reviewed by an experienced renal pathologist and biopsies re-scored using the ISN/RPS classification and NIH activity and chronicity indices. Distinct histological features were assessed individually (e.g. TMA). Persistent C3 hypocomplementemia was defined as decreased serum C3 levels at the time of biopsy and after 6 months (i.e. after the completion of induction), with concurrent normal serum C4 levels at 6 months. Early renal recovery was defined as either an increase in eGFR above 60 in those with a baseline eGFR &lt; 60 ml/min/1.73 m2, or a 50% decrease in proteinuria in those with a baseline eGFR ≥ 60 ml/min/1.73 m2 and ≥ 0.5 g/24-h or g/g UPCR at the time of biopsy. Variables were tested for their predictive power of death-censored ESRD in univariate and multivariate Cox regression models. Results 74 patients (NIAMS n = 36; Parma n = 38) met our criteria. Median follow-up duration was 64 months (range 6-230). On univariate analysis, the following parameters predicted ESRD: Hispanic ethnicity; age at biopsy; persistent C3 hypocomplementemia; normalization of both C3 and C4; renal recovery after induction; NIH activity index; presence of TMA; ≥ 25% IFTA. Multivariate Cox regression models for ESRD were created considering statistically significant variables (p &lt; 0.05). In a model including Hispanic ethnicity, age at biopsy, and persistent C3 hypocomplementemia, the latter predicted ESRD with an HR of 5.22 (95% CI [1.33, 20.58] p = 0.018) when adjusting for renal recovery after induction. Upon including histological features in the model, persistent C3 hypocomplementemia, TMA, and the NIH activity index lost significance, while ≥ 25% IFTA predicted ESRD with an HR of 27.26 (95% CI [2.12, 350.54], p = 0.011). Conclusion In patients with LN, ≥ 25% IFTA at baseline biopsy is a predictor of ESRD, allowing for early risk stratification with the potential of informing treatment strategies. Where percent IFTA is unavailable or its assessment unreliable (e.g. inadequate biopsy specimen for tubulointerstitial assessment), persistent C3 hypocomplementemia represents a reliable and reproducible early predictor of ESRD, irrespective of early renal recovery after induction.

scholarly journals Distribution and association of cancer with mortality in end-stage renal disease patients receiving dialysis

2019 ◽  
Vol 32 (6) ◽  
pp. 1003-1009
Author(s):  
Rajkumar Chinnadurai ◽  
Emma Flanagan ◽  
Philip A. Kalra
Keyword(s):  
Cox Regression ◽  
Matched Sample ◽  
Cancer History ◽  
Cancer Group ◽  
All Cause Mortality ◽  
History Of ◽  
Stage Renal Disease ◽  
End Stage ◽  
Esrd Patients

Abstract Background and aims Cancer in end-stage renal disease (ESRD) patients is an important comorbidity to be taken into consideration while planning for renal replacement therapy (RRT) options due to its associated increased mortality. This study aims to investigate the natural history and association of cancer with all-cause mortality in an ESRD population receiving dialysis. Method The study was conducted on 1271 ESRD patients receiving dialysis between January 2012 and December 2017. A comparative analysis was carried out between 119 patients with and 1152 without cancer history at entry into this study (baseline). A 1:2 (119 cancer: 238 no cancer) propensity score matched sample of 357 patients was also used for analysis. Cox-regression analysis was used to study the strength of the association between cancer and all-cause mortality. Kaplan–Meier (KM) analysis was used to demonstrate the difference in cumulative survival between the groups. A competing risk analysis was also carried out to calculate the probability of competing events (death, transplant and incident cancer). Results At baseline, 10.1% of the cohort had a history of cancer (current and past) with the annual incident rate being 1.3%. Urological cancers were the leading site of cancer. The median age of our cohort was 63 years with a predominance of males (63%) and Caucasians (79%). The majority (69%) of the cohort were receiving haemodialysis. 47% had a history of diabetes with 88% being hypertensive. During a median follow-up of 28 months, the proportion of deaths observed was similar between the groups in the matched sample (cancer 49.6 versus no-cancer 52.1%, p value 0.77). In a univariable Cox-regression model, there was no significant association between cancer and all-cause mortality (HR 1.28; 95% CI 0.97–1.67; p = 0.07). The KM estimates showed similar observations in the cumulative survival between the groups (matched sample log-rank, p value 0.85). In competing risk analysis, the cumulative probability of death at 5 years was non-significantly higher in the cancer group (cancer group 64% vs no cancer group 51%, p value 0.16). Conclusions In our real-world multi-morbid dialysis cohort of 119 cancer patients, baseline cancer history did not prove to be an independent risk factor for all-cause mortality in the first 5 years of follow-up, suggesting the need for a case-by-case approach in provision of RRT options, including transplantation.

Long Term Follow-up of Recipients of Combined HLA-Matched Nonmyeloablative Bone Marrow and Kidney Transplantation for Multiple Myeloma with End-Stage Renal Disease.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 3368-3368
Author(s):  
Thomas R Spitzer ◽  
Megan Sykes ◽  
Nina Tolkoff Rubin ◽  
Tatsuo Kawai ◽  
Steven L McAfee ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Renal Function ◽  
Normal Renal Function ◽  
End Stage Renal Disease ◽  
Renal Allograft ◽  
Chronic Gvhd ◽  
Mixed Chimerism ◽  
Stage Renal Disease ◽  
End Stage

Abstract Abstract 3368 Poster Board III-256 Specific tolerance following combined kidney and bone marrow transplantation (Kd/BMT)for patients with end stage renal disease (ESRD) with or without an underlying malignancy has been accomplished, as evidenced by prolonged normal renal function without ongoing immunosuppression (IS)and the demonstration of in vitro donor specific hyporesponsiveness (N Engl J Med 2008; 358:353-361; Am J Transplant 2006;6:2121-2133). In order to achieve potent anti-myeloma responses and induce tolerance through the induction of mixed chimerism (MC) for the renal allograft, 7 patients (median age 48 (34-55) yrs with multiple myeloma (MM) and ESRD received a combined HLA-matched Kd/BMT, with longest follow-up time of almost 11 years. An eighth pt developed cyclophosphamide (CY) cardiotoxicity on day -5 and did not receive a transplant. Preparative therapy for the transplants consisted of CY 60 mg/kg (days -5, -4) with hemodialysis 14 hrs after each CY dose, equine anti-thymocyte globulin, 15-20 mg/kg on days -1, +1, +3, and +5 and thymic irradiation (700 cGy) on day -1. Cyclosporine (CSP) was begun on day -1, with combined Kd/BMT on day 0. Nine additional donor lymphocyte infusions were given to 5 pts (5 for chimerism conversion, 4 for persistent/progressive disease (PD)). Acute (A) and chronic (C) GVHD developed in one patient following DLI for early PD, while chronic GVHD developed in two pts (one after a second stem cell transplant). Characteristics and outcomes of the 7 combined Kd/BMT recipients are as follows: # after 2nd transplant (myeloablative) from the same donor ; FDC: full donor chimerism In summary, 5 of 7 pts are alive, 4 without evidence of MM from 2.7 to 10.9 yrs post-Kd /BMT. Three pts have normal renal function without IS, while two pts have normal renal function on IS for chronic GVHD. Sustained renal allograft tolerance and prolonged anti-myeloma responses are achievable following nonmyeloablative HLA-matched kidney and BMT and the induction of mixed chimerism. This study was supported by the Immune Tolerance Network, National Institute of Allergy and Infectious Diseases. Disclosures: Off Label Use: Equine anti-thymocyte globulin: in vivo T cell depletion Cyclophosphamide:conditioning therapy for transplantation.

scholarly journals Treatment Outcomes of Anti-Neutrophil Cytoplasmic Autoantibody-Associated Vasculitis in Patients Over Age 75 Years: A Meta-Analysis

2020 ◽  
Vol 51 (4) ◽  
pp. 327-336
Author(s):  
Adam D. Morris ◽  
Mohamed E. Elsayed ◽  
Arvind Ponnusamy ◽  
Anthony Rowbottom ◽  
Francis Martin ◽  
...  
Keyword(s):  
Systematic Review ◽  
Cox Regression ◽  
Meta Analysis ◽  
Significant Risk ◽  
Limiting Factor ◽  
Significant Survival ◽  
Stage Renal Disease ◽  
End Stage ◽  
Immunosuppression Therapy

Background: The benefits of treating anti-neutrophil cytoplasmic autoantibody-associated vasculitis (AAV) in advancing age remains unclear with most published studies defining elderly as ≥65 years. This study aims to determine outcomes of induction immunosuppression in patients aged ≥75 years. Methods: A cohort of patients aged ≥75 years with a diagnosis of AAV between 2006 and 2018 was constructed from 2 centres. Follow-up was to 2 years or death. Analysis included multivariable Cox regression to compare mortality and end-stage renal disease (ESRD) based on receipt of induction immunosuppression therapy with either cyclophosphamide or rituximab. A systematic review of outcome studies was subsequently undertaken amongst this patient group through Pubmed, Cochrane and Embase databases from inception until October 16, 2019. Results: Sixty-seven patients were identified. Mean age was 79 ± 2.9 years and 82% (n = 55) received induction immunosuppression. Following systematic review, 4 studies were eligible for inclusion, yielding a combined total of 290 patients inclusive of our cohort. The aggregated 1-year mortality irrespective of treatment was 31% (95% CI 25–36%). Within our cohort, induction immunosuppression therapy was associated with a significantly lower 2-year mortality risk (hazard ratio [HR] 0.29 [95% CI 0.09–0.93]). The pooled HR by meta-analysis confirmed this with a significant risk reduction for death (HR 0.31 [95% CI 0.16–0.57], I2 = 0%). Treated patients had a lower pooled rate of ESRD, but was not statistically significant (HR 0.71 [95% CI 0.15–3.35]). Conclusion: This meta-analysis suggests that patients ≥75 years with AAV do benefit from induction immunosuppression with a significant survival benefit. Age alone should not be a limiting factor when considering treatment.

Nierenbeteiligung bei ANCA-assoziierten Vaskulitiden

2018 ◽  
Vol 143 (02) ◽  
pp. 79-88 ◽  
Author(s):  
Marion Haubitz
Keyword(s):  
Renal Function ◽  
Maintenance Treatment ◽  
Renal Involvement ◽  
Young Patients ◽  
Induction Treatment ◽  
Renal Manifestation ◽  
Increased Risk ◽  
Stage Renal Disease ◽  
End Stage

AbstractIn patients with ANCA-associated vasculitis renal involvement is frequently seen and the severity of renal manifestation is very important for therapeutic strategies and prognosis. Clinically rapid loss of renal function, nephritic sediment and proteinuria in a non-nephrotic range are characterizing a focal segmental necrotizing pauci-immune glomerulonephritis with extrarenal proliferations. Induction treatment depends on the severity of manifestations. With a normal renal function methotrexate can be used in combination with steroids. In patients with organ threatening involvement but creatinine below 500 µmol/l cyclophosphamide pulses or Rituximab should be used together with steroids, initially with i. v. pulses. Rituximab is more effective in PR3-ANCA vasculitis and should be used in relapsing disease, in young patients to avoid gonadal toxicity and in patients with an increased risk of malignancies. In patients on dialysis or with creatinine > 500 µmol/l plasma exchange should be added. Maintenance treatment (mainly with azathioprine) is necessary as at least 50 % of the patients develop relapses. Rituximab seems more effective, however it is not approved for maintenance treatment and no long-term data are available. Adjuvant treatment, long-term side effects and the increased incidence of cardiovascular events have to be included in the follow-up of vasculitis patients. In end-stage renal disease patients relapses occur but are more difficult to diagnose and treat with higher incidence of infections. Transplantation should be offered as patient and transplant survival is good.

Plasma Exchange Treatment in Rapidly Progressive Glomerulonephritis Associated with Antineutrophil Cytoplasmic Autoantibodies

1992 ◽  
Vol 15 (3) ◽  
pp. 181-184 ◽  
Author(s):  
G.M. Frascà ◽  
N.G. Zoumparidis ◽  
L.C. Borgnino ◽  
L. Neri ◽  
A. Vangelista ◽  
...  
Keyword(s):  
Renal Function ◽  
Disease Activity ◽  
Plasma Exchange ◽  
Systemic Disease ◽  
Rapidly Progressive Glomerulonephritis ◽  
Risk Of Death ◽  
Systemic Symptoms ◽  
Stage Renal Disease ◽  
End Stage ◽  
Antineutrophil Cytoplasmic Autoantibodies

This study reports on 12 patients with acute renal failure due to biopsy-proven rapidly progressive glomerulonephritis and signs of systemic disease in whom antineutrophil cytoplasmic autoantibodies (ANCA) were detected by indirect immunofluorescence (IIF) on alcohol-fixed neutrophils and assessed in serial determinations by ELISA. The diagnosis was: Wegener's granulomatosis in nine patients who showed a diffuse cytoplasmic pattern at IIF (c-ANCA), and microscopic polyarteritis in three where a perinuclear pattern (pANCA) was seen. All patients underwent a course of plasma exchange - PE - (3-10 sessions per patient) associated with steroids and cyclophosphamide. The ANCA titer dropped steeply during PE in all cases and was followed by disappearance of systemic symptoms and renal function improvement within four weeks. After a follow-up period of 50 ± 31.2 months all patients were alive without signs of disease activity; ten had stable renal function, with serum creatinine 1.8 ± 0.7 mg/dl; two had entered regular dialysis treatment after 44 and 82 months. Our results suggest that the rapid removal of ANCA by means of PE can help control disease activity and reduce the risk of death or end-stage renal disease.

scholarly journals Fibrillary glomerulonephritis with a favourable prognosis of 26 years

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.

scholarly journals Histopathological Classification—A Prognostic Tool for Rapidly Progressive Glomerulonephritis

Medicina ◽  
2018 ◽  
Vol 54 (2) ◽  
pp. 17 ◽  
Author(s):  
Marta Kantauskaitė ◽  
Agnė Laučytė-Cibulskienė ◽  
Marius Miglinas
Keyword(s):  
Cox Regression ◽  
Rapidly Progressive Glomerulonephritis ◽  
Glomerular Sclerosis ◽  
Histopathological Classification ◽  
Prognostic Effect ◽  
Autoimmune Antibodies ◽  
Renal Biopsies ◽  
One Year ◽  
Stage Renal Disease ◽  
End Stage

Background: Recently proposed histopathological classification may predict patient outcome in pauci-immune glomerulonephritis. This study sought to prove that the prognostic effect could be extended to all types of rapidly progressive glomerulonephritis. Methods: Retrospective analysis of patients diagnosed with rapidly progressive glomerulonephritis between April 1999 and August 2015 was performed. Epidemiological and clinical data were collected from medical records. The descriptions of renal biopsies were reviewed and classified into focal, sclerotic, crescentic and mixed class according to classification proposed by Berden et al. The study end points were end stage renal disease (ESRD) or death. Survival analyses were modelled using Cox regression. Results: 73 renal biopsies with diagnosis of rapidly progressive glomerulonephritis were included in the study. 25 (34.2%), 16 (21.9%), 24 (32.9%) and 8 (11%) patients were assigned to focal, crescentic, mixed and sclerotic class, respectively. Thirty-two (42.5%) patients were anti-neutrophil cytoplasmic antibody (ANCA) negative, of which eight (10.9%) were anti–glomerular basement membrane antibody (anti–GBM) positive and 24 (32.8%) were negative for autoimmune antibodies. Six (8.2%) patients died within one year. Among patients who survived, median change in estimated glomerular filtration rate (eGFR) values were: −10.5 mL/min in focal, 4.2 mL/min in crescentic, −4.3 mL/min in mixed and 4.1 mL/min in sclerotic group, p > 0.05. In the Cox regression model, there was no significant predictor of patient survival whereas the sclerotic group (HR 3.679, 95% CI, 1.164–11.628, p < 0.05) and baseline eGFR of <15 mL/min (HR 4.832, 95% CI, 1.55–15.08, p < 0.01) had an unfavorable effect for renal survival. Conclusions: Predominant glomerular sclerosis and low eGFR at baseline are associated with higher risk of ESRD in cases with crescentic glomerulonephritis. Therefore, despite the origin of injury, histological classification might aid in prediction of patient outcomes in rapidly progressive glomerulonephritis.

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