scholarly journals Clinicopathological Patterns and Predictors of the Functional Restoration of Immunoglobulin G4-Related Kidney Disease: A Chinese Single-Center Cohort Study

2021 ◽  
Vol 8 ◽  
Author(s):  
Tao Su ◽  
Hui Wang ◽  
Suxia Wang ◽  
Li Yang
Keyword(s):  
Regression Analysis ◽  
Kidney Disease ◽  
Immunosuppressive Therapy ◽  
Kidney Biopsy ◽  
Renal Outcome ◽  
Clinicopathological Parameters ◽  
Disease Relapse ◽  
Immunoglobulin G4 ◽  
Interstitial Inflammation

Background: Immunoglobulin G4-related disease (IgG4-RD) is a systemic immunoreactivity-based fibro-inflammatory disease. Immunoglobulin G4-related kidney disease (IgG4-RKD) is a frequently overlooked diagnosis. This study aimed to describe IgG4-RKD and examine the factors relevant to the renal outcomes of IgG4-RD.Methods: We studied a prospective IgG4-RKD cohort between January 2012 and December 2020 with close follow-up. Clinicopathologic data at kidney biopsy were collected and analyzed. We aimed to explore independent risk factors for long-term renal outcome and disease relapse. Patients with an eGFR<45 ml/min per 1.73m2 at 12 months were defined as having poor outcomes.Results: The included 42 patients with IgG4-RKD had a mean age of 58.5 ± 8.7 years (male-to-female ratio = 5:1). The IgG4-RD responder index (RI) was 12.2 ± 3.3. A total of 66.7% of the patients presented with acute on kidney disease or acute on chronic kidney disease. Eight patients (19.0%) showed nephrotic-range proteinuria, and nine (21.4%) had high-titer IgG4-autoantibodies, including antineutrophil cytoplasmic antibody and anti-phospholipase A2 receptor. A kidney biopsy was conducted in 40 patients. Thirty-seven (90.0%) patients were diagnosed with IgG4-related tubulointerstitial nephritis, and 19 (47.5%) of them had concurrent glomerular diseases (membranous nephropathy [MN], n = 3; crescentic glomerulonephritis [CrGN], n = 11; diabetic kidney disease, n = 3; and both MN and CrGN, n = 2). IgG4-RD RI had a close relationship with serum C3 (R = −0.509, P = 0.001), C4 (R = −0.314, P = 0.049) levels, and peripheral blood eosinophil count (PBEC; R = 0.377, P = 0.024), factors that were not included in RI scores. Correlation analysis disclosed that IgG4-RD RI (R = 0.422, P = 0.007), organs involved (R = 0.452, P = 0.003), and C3 (R = −0.487, R = 0.002) were correlated with the percentage decrease of serum creatinine at 1 month. However, multivariate regression analysis failed to identify any clinicopathological parameters that could predict short-term renal restoration and IgG4-RKD relapse. Ten out of 29 variables, of most importance, were identified by the least absolute shrinkage and selection operator (LASSO) regression analysis. By multivariate logistic regression a higher serum IgG4 (OR = 0.671, P = 0.010), IgG1 (OR = 1.396, P = 0.049), IgG3 (OR = 19.154, P = 0.039), and erythrocyte sedimentation rate (ESR; OR = 1.042, P = 0.032) were found to be independent factors for poor long-term outcome. Conventional immunosuppressive medications and/or rituximab were prescribed, and in 83.3% of the patients, the kidney function improved. Repeat kidney biopsies confirmed the remission of interstitial inflammation in two patients under immunosuppressive therapy. However, the disease relapse rate was as high as 31.0%.Conclusions: We strongly recommend a kidney biopsy in active IgG4-RD, especially when there is proteinuria and renal dysfunction, because concurrent glomerular involvement and active interstitial inflammation should be assessed. A higher serum IgG1, IgG3, and ESR were independent factors for the poor long-term renal outcome; however, elevated IgG4 predicted a good renal prognosis, and appropriate and timely immunosuppressive therapy can help achieve a better prognosis.

ANCA-associated vasculitis with dominant renal involvement: clinical and morphological presentation and outcomes

2019 ◽  
Vol 23 (6) ◽  
pp. 29-44
Author(s):  
V. A. Dobronravov ◽  
A. V. Karunnaya ◽  
A. V. Kazimirchik ◽  
A. V. Smirnov
Keyword(s):  
Kidney Disease ◽  
Serum Creatinine ◽  
Clinical Remission ◽  
Kidney Biopsy ◽  
Morphological Parameters ◽  
Baseline Serum ◽  
Interstitial Inflammation ◽  
Baseline Serum Creatinine

THE AIM: The analysis of clinical and morphological presentations and outcomes of primary systemic vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA-V) with dominant kidney involvement; the determination of clinical and morphological parameters associated with prognosis.PATIENTS AND METHODS. Eighty nine patients with morphologically confirmed ANCA-associated kidney vasculitis on standard immunosuppressive therapy (IST) were included in this retrospective study. Clinical, immunological, and histological indices were analyzed at the time of the kidney biopsy, and early in the short-term (3-6 months) and in the long-term follow-up. The following outcomes were evaluated: the achievement of clinical and immunological remission of the disease; eGFR at the end of follow-up, the progression of renal disease (by the composite point: initiation of renal replacement therapy (RRT) or the estimated glomerular filtration rate (eGFR) <15 ml/min/1.73m2 or decrease in eGFR >50 %); all-cause mortality. The prognostic significance of clinical and morphological parameters was evaluated in multivariable regression models.RESULTS. Most of cases (78 %) were represented by rapidly progressive or acute nephritic syndrome. Mean eGFR was 23 ml/min/1.73 m2. Fifteen percent of patients required acute dialysis. Dominant morphological phenotypes of glomerular lesions were sclerotic (34 %) and mixed (36 %) according to the International Pathology Classification (IPC). Median follow-up was 24 (8; 55) months. Cumulative 5-year and 10-year patient’s survivals and were 92 % and 86 %, respectively. Cumulative 5-year and 10-year renal survivals were 86 % and 68 %, respectively. The cumulative 5-year and 10-year proportions of cases without progression of kidney disease were 80 % and 55 %, respectively. Within 3-6 months of the induction IST 81 % of patients achieved clinical remission (complete (59 %) or partial (22 %)) (CR3-6), while 84 % of patients had immunological remission. Serum creatinine (Pcr) at the time of kidney biopsy was only the factor associated with the risk of renal progression (Expβ=1.73 (95 %CI 1.40-2.14) per 0.1 mmol/l increase). IPC classes and ANCA Renal Risk Score (ARRS) groups as well as other morphological indices of kidney injury had no independent associations with the renal outcomes in Cox models adjusted for Pcr. The independent factors associated with eGFR at the end of follow-up were: CR3-6 (β=0.36±0.08, p<0.001); age (β=-0.34±0.09, p<0.001), Pcr (β=-0.35±0.09, p<0.001) and the global glomerulosclerosis (β=0.28±0.08, p<0.001). CR3-6 (β=0.57±0.10, p<0.001), and the proportion of cellular crescents (β=0.26±0.12, p=0.023) and interstitial inflammation (β=0.27±0.11, p=0.026) were also independently associated with the change of eGFR by the end of follow-up.CONCLUSION. An unfavorable renal prognosis for ANCA-V determined by severe renal dysfunction due to inflammatory and fibrotic alterations of the organ can be significantly improved by adequate therapy with the achievement of higher patient’s and kidney’s survival. The baseline serum creatinine is only the factor associated with the long-term risks of dialysis and kidney disease progression. In addition to baseline serum creatinine and the development of early clinical remission, the separate assessment of global glomerular sclerosis, cellular crescents, and interstitial inflammation may be more useful for the individual prediction of long-term eGFR changes than IPC classes or ARRS.

scholarly journals Different Patterns of Kidney Fibrosis Are Indicative of Injury to Distinct Renal Compartments

Cells ◽  
2021 ◽  
Vol 10 (8) ◽  
pp. 2014
Author(s):  
Désirée Tampe ◽  
Laura Schridde ◽  
Peter Korsten ◽  
Philipp Ströbel ◽  
Michael Zeisberg ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Renal Outcome ◽  
Diffuse Fibrosis ◽  
Tubular Atrophy ◽  
Kidney Fibrosis ◽  
Interstitial Inflammation ◽  
Tubulointerstitial Lesions ◽  
Nephron Loss ◽  
Glomerular Damage

Kidney fibrosis is a common manifestation and hallmark of a wide variety of chronic kidney disease (CKD) that appears in different morphological patterns, suggesting distinct pathogenic causes. Broad macroscopically visible scars are the sequelae of severe focal injury and complete parenchymal destruction, reflecting a wound healing response as a consequence of infarction. In the kidney, chronic glomerular injury leads to atrophy of the corresponding tubule, degeneration of this specific nephron, and finally interstitial fibrosis/tubular atrophy (IF/TA). Compared to this glomerulus-induced focal replacement scar, diffuse fibrosis independent of tubular atrophy appears to be a different pathogenic process. Kidney fibrosis appears to develop in a compartment-specific manner, but whether focal and diffuse fibrosis has distinct characteristics associated with other glomerular or tubulointerstitial lesions remains elusive. In the present study, we aimed to analyze renal fibrotic patterns related to renal lesions, which directly contribute to renal fibrogenesis, to unravel fibrotic patterns and manifestations upon damage to distinct renal compartments. Patterns of kidney fibrosis were analyzed in experimental models of CKD and various renal pathologies in correlation with histopathological and ultrastructural findings. After the induction of isolated crescentic glomerulonephritis (GN) in nephrotoxic serum-nephritis (NTN), chronic glomerular damage resulted in predominantly focal fibrosis adjacent to atrophic tubules. By contrast, using unilateral ureteral obstruction (UUO) as a model of primary injury to the tubulointerstitial compartment revealed diffuse fibrosis as the predominant pattern of chronic lesions. Finally, folic acid-induced nephropathy (FAN) as a model of primary tubular injury with consecutive tubular atrophy independent of chronic glomerular damage equally induced predominant focal IF/TA. By analyzing several renal pathologies, our data also suggest that focal and diffuse fibrosis appear to contribute as chronic lesions in the majority of human renal disease, mainly being present in antineutrophil cytoplasmic antibody (ANCA)-associated GN, lupus nephritis, and IgA nephropathy (IgAN). Focal IF/TA correlated with glomerular damage and irreversible injury to nephrons, whereas diffuse fibrosis in ANCA GN was associated explicitly with interstitial inflammation independent of glomerular damage and nephron loss. Ultrastructural analysis of focal IF/TA versus diffuse fibrosis revealed distinct matrix compositions, further supported by different collagen signatures in transcriptome datasets. With regard to long-term renal outcome, only the extent of focal IF/TA correlated with the development of end-stage kidney disease (ESKD) in ANCA GN. In contrast, diffuse kidney fibrosis did not associate with the long-term renal outcome. In conclusion, we here provide evidence that a focal pattern of kidney fibrosis seems to be associated with nephron loss and replacement scarring. In contrast, a diffuse pattern of kidney fibrosis appears to result from primary interstitial inflammation and injury.

scholarly journals MO638ANEMIA IN DIABETIC PATIENTS REFLECTS SEVERE TUBULOINTERSTITIAL INJURY AND ASSISTS CLINICALLY IN PREDICTING DIAGNOSIS OF DIABETIC NEPHROPATHY

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Soichiro Yokota ◽  
Kenji Ito ◽  
Maho Watanabe ◽  
Koji Takahashi ◽  
Naoko Himuro ◽  
...  
Keyword(s):  
Renal Function ◽  
Regression Analysis ◽  
Diabetic Nephropathy ◽  
Kidney Disease ◽  
Confidence Interval ◽  
Odds Ratio ◽  
Kidney Biopsy ◽  
Renal Pathology ◽  
Diabetic Patients ◽  
Pathological Findings

Abstract Background and Aims Diabetic nephropathy (DN) is currently a leading cause of end-stage kidney disease worldwide. Kidney biopsy is generally performed in diabetic patients to discriminate between DN and non-diabetic kidney disease (NDKD), and to provide more specific treatments. In addition to conventional predicting factors of DN, recent studies suggested the predictive value of anemia in the diagnosis of DN, however detailed pathophysiology and the significance of anemia in renal pathology are not fully understood. This study aimed to investigate the impact of anemia on renal pathology and clinical course in patients who underwent kidney biopsy. Method We reviewed 81 patients (60.4 ± 13.7 years, 54 men and 27 women) with type 2 diabetes who underwent percutaneous kidney biopsy in Fukuoka University Hospital from January 2001 through March 2020. DN was diagnosed by mesangial expansion or nodular glomerulosclerosis observed under a light microscope, and immunofluorescence assisted in differentiating NDKD from DN. Anemia was defined as hemoglobin level &lt;13 g/dL in males and &lt;12 g/dL in females in accordance with the World Health Organization standards. Laboratory and pathological findings, and clinical courses were investigated. Results According to their pathological findings, patients were classified into two groups: isolated DN (DN group, n=30) and NDKD alone or concurrent DN (NDKD group, n=51). There were 11 types of NDKD. Of these, membranous nephropathy was the most common (23.5%), followed by IgA nephropathy (17.6%), and crescentic glomerulonephritis (13.7%). In multiple logistic regression analysis, absence of severe hematuria (odds ratio (OR) 11.66, 95% confidence interval (CI) 1.68 - 89.9) and presence of anemia (OR 11.38, 95% CI 2.51 - 51.52) were significantly related with the diagnosis of DN. Akaike’s information criterion (AIC) and net reclassification improvement (NRI) analyses revealed improved predictive performance by adding anemia to the conventional factors (AIC 100.152 to 91.844; NRI 27.0%). The tissues of patients in the DN group demonstrated more severe interstitial fibrosis and tubular atrophy (IF/TA) than the NDKD group (p&lt;0.05) regardless of the rate of global glomerulosclerosis (figure), and IF/TA was related to the prevalence of anemia (odds ratio: 7.31, 95% confidence interval: 2.33 - 23.00) in multivariate regression analysis. These results suggest DM-associated severe IF/TA (compared with NDKD) impaired erythropoietin production, resulting in earlier anemia, independent of glomerular injuries and renal function. Furthermore, the renal prognosis was significantly better in the NDKD group than in the DN group using Log-rank test (p&lt;0.05). Conclusion DN is associated with anemia because of severe IF/TA regardless of renal function, and anemia helps clinician discriminate clinically between isolated DN and NDKD.

scholarly journals MP352PREDICTORS OF LONG TERM RENAL OUTCOME IN PREGNANT WOMEN WITH CHRONIC KIDNEY DISEASE ATTENDING A COMBINED REGIONAL RENAL OBSTETRIC SERVICE

2017 ◽  
Vol 32 (suppl_3) ◽  
pp. iii556-iii556
Author(s):  
Monisha Chakravorty ◽  
Nadia Sarween ◽  
Jemma Proudfoot-Jones ◽  
Rachel Clements ◽  
Ellen Knox ◽  
...  
Keyword(s):  
Chronic Kidney Disease ◽  
Kidney Disease ◽  
Pregnant Women ◽  
Renal Outcome

scholarly journals Focal Segmental Glomerulosclerosis, Risk Factors for End Stage Kidney Disease, and Response to Immunosuppression

Kidney360 ◽  
2020 ◽  
pp. 10.34067/KID.0006172020
Author(s):  
Benjamin M. Forster ◽  
Robert Nee ◽  
Dustin J. Little ◽  
Peter J. Greasley ◽  
James B. Hughes ◽  
...  
Keyword(s):  
Risk Factors ◽  
Kidney Disease ◽  
Focal Segmental Glomerulosclerosis ◽  
Renal Survival ◽  
End Stage Kidney Disease ◽  
Interstitial Inflammation ◽  
Segmental Glomerulosclerosis ◽  
End Stage

Background: Focal segmental glomerulosclerosis (FSGS) is a heterogeneic glomerular disease. Risk factors for end- stage kidney disease (ESKD) and impact of immunosuppression treatment (IST) has varied in previously published cohorts. These cohorts were limited by relatively small case numbers, short follow up, lack of racial/ethnic diversity, a mix of adult and pediatric patients, lack of RAAS inhibition, or lack of subgroup analysis of IST. Methods: We compared demographics, clinical characteristics, histopathology and IST to long term renal survival in a large, ethnically diverse, adult cohort of 338 biopsy-proven FSGS cases with long term follow up in the era of RAAS inhibition using data from the United States Department of Defense health care network. Results: Multivariate analysis showed that nephrotic range proteinuria (NRP), estimated glomerular filtration rate <60 ml/min/1.73m2, hypoalbuminemia, interstitial fibrosis and tubular atrophy, and interstitial inflammation at diagnosis as well as the absence of remission were all associated with worse long term renal survival. IgM, C3, and a combination of IgM/C3 immunofluorescence staining were not associated with reduced renal survival. IST was not associated with improved renal survival in the whole cohort, or in a subgroup with NRP. However, IST was associated with better renal survival in a subgroup of FSGS cases with both NRP and hypoalbuminemia and hypoalbuminemia alone. Conclusion: Our study suggests that IST should be reserved for FSGS patients with nephrotic syndrome. It also introduces interstitial inflammation as a potential risk factor for ESKD and does not support the proposed pathogenicity of IgM and complement activation.

P0383A LONG-TERM RETROSPECTIVE OUTCOME ANALYSIS OF ANCA-NEGATIVE PAUCI-IMMUNE GLOMERULONEPHRITIS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Jennifer O'Brien ◽  
Pek Ghe Tan ◽  
Charles Pusey ◽  
Stephen McAdoo
Keyword(s):  
Overall Survival ◽  
Kidney Disease ◽  
Immunosuppressive Therapy ◽  
Systemic Disease ◽  
P Value ◽  
Negative Group ◽  
End Stage Kidney Disease ◽  
Rate Of Progression ◽  
End Stage

Abstract Background and Aims Pauci-immune glomerulonephritis (GN), usually associated with circulating antineutrophil cytoplasm antibodies (ANCA), is one of the most common causes of rapidly progressive glomerulonephritis that results in high incidence of end-stage kidney disease (ESKD). Most of the existing large trials looking at treatment efficacies exclude ANCA-negative patients, and relatively few studies have reported their long-term outcomes. Therefore, we conducted a single-centre retrospective study to examine the long-term overall survival and renal outcome in this cohort of patients. Method All cases of newly diagnosed biopsy-proven pauci-immune GN from 2006 - 2019 were identified through a local histopathology database. Patients with negative anti-myeloperoxidase (MPO) and anti-proteinase-3 (PR3) serology were identified (ANCA-negative group) and comparisons made with the cohort of patients with positive serology (MPO/PR3 positive group). Patients with relapsing ANCA-GN, eosinophilic granulomatosis with polyangitis, other co-existing glomerulonephritis or missing data on induction therapy or outcome were excluded. Baseline demographics, initial serum creatinine (sCr), estimated glomerular filtration rate (eGFR), systemic involvement and histopathology features including percentage normal glomeruli, and interstitial fibrosis/tubular atrophy score of &gt;25% were collected and compared. Kaplan Meier survival analysis was used to compare overall survival and end-stage kidney disease (ESKD) progression rate between the two groups. Results 178 patients were identified with a median follow-up of 44 months. 83 were female (47%) and median age was 62 years. 15 (8%) were ANCA-negative. 163 (92%) were MPO- and/or PR3-ANCA positive. There were no differences in baseline characteristics such as age, gender and proportion of patients with normal glomeruli &lt;25% on initial histology. However, we observed a significantly higher proportion of patients with renal-limited vasculitis in the ANCA-negative group (67% vs 24% p=0.01) and more severe renal dysfunction at presentation (median sCr 309umol/L vs 204umol/L, p= 0.01). We also demonstrated a higher proportion of patients with an IFTA score of &gt;25% on renal biopsy (53% with &gt;25% IFTA in ANCA-negative cohort vs 27%, p =0.03). The ANCA-negative group were more likely to receive combination immunosuppressive therapy that included plasma exchange (47% vs 23%, p value 0.04). When considering overall survival there was significantly higher mortality (40% vs 16%, p value 0.009) and rate of progression to ESKD (53% vs 18%, p value 0.001) in the ANCA-negative group as a whole. When we compared patients with renal-limited vasculitis only however, there was no significant difference in either overall survival or rate of progression to ESKD (p=0.85 and 0.84 respectively). We found that ANCA-negative patients with systemic disease did still have significantly higher rates of both progression to ESKD and overall mortality (p=0.002 and p=0.02 respectively). Conclusion In our cohort, patients with ANCA-negative pauci-immune GN have poorer renal function and higher IFTA scores on biopsy at presentation perhaps reflecting delayed diagnosis due to a lack of diagnostic serology and the higher proportion of renal-limited disease in this subgroup. Despite intensive immunosuppressive therapy, this study observed overall higher treatment failure rates in ANCA-negative patients, largely in those with systemic disease. This possibly relates to a different underlying disease process. Larger, prospective studies are required to enhance understanding of the disease pathogenesis to allow optimal tailored treatment for these patients.

scholarly journals FP151THE LONG-TERM RENAL OUTCOME IN PATIENTS WITH ANCA-ASSOCIATED VASCULITIS: PREDICTIVE VALUE OF KIDNEY BIOPSY

2015 ◽  
Vol 30 (suppl_3) ◽  
pp. iii117-iii117
Author(s):  
Ludmila Biriukova ◽  
Ekaterina Stolyarevich ◽  
Nadia Frolova ◽  
Ludmila Artyukhina ◽  
Natalia Tomilina
Keyword(s):  
Predictive Value ◽  
Kidney Biopsy ◽  
Renal Outcome ◽  
Anca Associated Vasculitis

scholarly journals Early response to immunosuppressive therapy predicts good renal outcome in lupus nephritis: Lessons from long-term followup of patients in the Euro-Lupus Nephritis Trial

2004 ◽  
Vol 50 (12) ◽  
pp. 3934-3940 ◽  
Author(s):  
Frédéric A. Houssiau ◽  
Carlos Vasconcelos ◽  
David D'Cruz ◽  
Gian Domenico Sebastiani ◽  
Enrique de Ramon Garrido ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Immunosuppressive Therapy ◽  
Early Response ◽  
Renal Outcome

Renal Involvement and Outcome In Monoclonal Lightchain Disease

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4985-4985
Author(s):  
Raoul Bergner ◽  
Michael J. Uppenkamp ◽  
Martin Hoffmann
Keyword(s):  
Renal Function ◽  
Kidney Disease ◽  
Renal Disease ◽  
Light Chain ◽  
Kidney Biopsy ◽  
Renal Involvement ◽  
The Other ◽  
Renal Outcome ◽  
Al Amyloidosis ◽  
Light Chain Disease

Abstract Abstract 4985 Background: Renal involvement is very common in monoclonal light chain disease (mLCD). However, the types of renal disease are manifold. Also the prognosis and outcome is very different. We analysed the data and renal outcome of patients with monoclonal light chain disease and renal disease. Methods: We analysed the data of patients with monoclonal light chain disease, who underwent a kidney biopsy due to proteinuria and/or renal insufficiency of unclear origin, retrospectively. Data of renal function, proteinuria, the type of renal disease and the renal outcome were collected. The mLCD were subclassified in multiple myeloma (MM), AL-amyloidosis (ALA), monoclonal gammopathy of unclear significance (MGUS) and B-cell non hodgkin lymphona (B-NHL). The kidney biopsy findings were classified in cast nephropathy (CN), ALA, light chain deposit disease (LCDD) and other renal disease (ORD). Results: 88 patients were included in the analysis. 47 patients suffered from MM, 15 from systemic ALA, 21 from MGUS and 5 from B-NHL, respectively. In 17 patients the mLCD was not known before kidney biopsy but detected by typical kidney disease. Of the 47 patients with MM 24 had CN, 4 LCDD, 4 ALA and 15 ORD, respectively. All patients with ALA had renal amyloidosis except one, who had an IgA-glomerulonephritis. All patients with MGUS suffered from ORD only. Patients with B-NHL had CN one patient, LCDD one patient, ALA one patient and ORD two patients, respectively. The ORD were also associated with the mLCD in 21 cases (interstitial nephritis n=7, nephrocalcinosis n=7 and membranoproliferative glomerulonephritis n=4), the other patients had kidney disease independent from mLCD (e.g. diabetic nephropathy, focal segmental glomerulosclerosis, IgA-glomerulonephritits or nephroangiosclerosis). The mean follow up time was 20.4±24.8 month. Patients with CN had a significant worse renal function at time of kidney biopsy [serum creatinine [mg/dl]: CN 4.7±4.0; LCDD 3.36±1.38; ALA 1.46±1.0; ORD 2.0±2.3; p< 0.001 CN vs. ALA and ORD]. Patients with ALA had a significant greater proteinuria [g/d], than the other patients [CN 3.3±2.5; LCDD 1.7±0.9; ALA 5.6±5.2; ORD 2.1±1.9; p< 0.05 ALA vs. LCDD and CN; p<0.001 ORD vs. ALA]. 3.7 months after kidney biopsy 50% of patients with CN were on dialysis (HD). At 12 month 59% of patients with CN were on HD, compared to 37% of patients with ALA, 20% of patients with LCDD and 8% of patients with ORD (p=0.0004). Patients on HD had a significant worse survival compared to those without HD [50% survival 5.3 vs. 42 months; p=0.005]. Discussion: Our data demonstrate, that not all patients with mLCD suffered from a mLCD associated renal disease. The renal prognosis was very different between the types of renal disease. The worst renal outcome had patients with CN followed by patients with ALA. The best renal outcome had patients with ORD. Once on HD the survival is worse than without HD. Based on our data we would recommend to clarify the exact type of renal disease in all patients with mLCD and any evidence of renal disease by kidney biopsy. Disclosures: No relevant conflicts of interest to declare.

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