scholarly journals AB1234 MICRO-RNA 155 AND MIR-34A: POSSIBLE BIOMARKERS OF INFLAMMATORY BURDEN AND DISEASE ACTIVITY IN ANCA-ASSOCIATED VASCULITIS WITH RENAL INVOLVEMENT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1908.2-1908
Author(s):  
D. Bruno ◽  
P. G. Cerasuolo ◽  
C. Di Mario ◽  
S. L. Bosello ◽  
L. Gigante ◽  
...  
Keyword(s):  
Renal Function ◽  
Kidney Biopsy ◽  
Urine Analysis ◽  
Renal Involvement ◽  
Kidney Tissue ◽  
Tissue Expression ◽  
Micro Rna ◽  
Renal Outcome ◽  
Markers Of Inflammation ◽  
Inflammatory Burden

Background:Predicting clinical outcomes in ANCA-related glomerulonephritis remains a major challenge. To date, there is no reliable biomarker able to predict renal prognosis in patients with ANCA-associated vasculitis (AAV). Micro-RNA (miRNA) are non-coding RNAs involved in the fine tuning of immune cells biology and this epigenetic modulation associates with different phenotypes and prognosis in several diseases.Objectives:To investigate the expression of miR-155 and miR-34a in kidney biopsies of AAV patients according with renal outcome.Methods:Fifteen patients with AAV and renal involvement (mean age 63.0 ±13.3 years, disease duration 4.9±2.2 months), who underwent renal biopsy. Demographic, clinical and autoimmune parameters were recorded for each patient. Each kidney biopsy was classified according to the Berden Classification, Risk group (according to the ANCA Renal Risk Score) and the chronicity Classification of the Mayo Clinic’s proposed score.MiR-155 and miR-34a expression was investigated on kidney biopsy tissue using the miRNeasy FFPE kit (Qiagen). The quantitative expression of miR-155, miR-34a and housekeeping gene U1, used as control, was assessed by Real Time-PCR. MiR-155 and miR-34a expression was correlated with histopathological and clinical-laboratory parameters.Each patient was followed for 12 months and renal outcome was considered according toKDIGO CKDClassification. Markers of inflammation (ESR, CRP) and urine analysis data were recorded at baseline and after 12 months.Results:Six (40%) patients were p-ANCA positive and 9 (60%) c-ANCA positive. Eight patients (53%) also had pulmonary involvement. The mean baseline GFR was 30.7±28.8 ml/min/1.73 m2and 10 patients (66%) showed an active urinary sediment.At disease onset, the mean expression of miR-155 was 9.5±21.1, while the expression of mir-34a was 13.1±46.2. Considering the autoimmune profile, kidney tissue of p-ANCA positive patients was enriched of mir-155 (19.6±30.6 fold) compared to c-ANCA positive patients (1.9±2.9 fold; p=0.001). Particularly, considering the renal function, kidney tissue of patients with greater impairment of renal function (KDIGO stage 5) was enriched of miR-155 (21.5±38.3 fold) compared to patients with less renal impairment (KDIGO stage 1-4) (4.72±8.16 fold, p=0.004).Tissue expression of miR-155 and miR-34a did not correlated with the abovementioned histopathological classifications.After 12 months from kidney biopsy, 3(20%) patients had a worsening of renal function, 5 (33%) still presented elevated markers of inflammation and 3 (20%) still had proteinuria at urine analysis. At baseline, kidney tissue of patients with higher CRP plasma levels and proteinuria at follow-up presented higher expression of miR-155 (p=0.002 and p=0.001), whereas no significant differences were found about miR-34a kidney tissue expression.Conclusion:MiRNAs may play a potential role in the pathogenesis of ANCA-related glomerulonephritis. MiR-155 kidney enrichment seems to mirror the disease inflammatory burden and activity at the onset and after 12 months representing a possible biomarker in ANCA vasculitis with renal involvement. This finding may represent the basis for further studies on miRNA expression in blood samples, aiming to identify a non-invasive biomarker of kidney damage, predicting disease’s relapses and patients’ prognosis.References:[1]Renauer et al, Clin Rev Allergy Immunol. 2016Disclosure of Interests:Dario Bruno: None declared, Pier Giacomo Cerasuolo: None declared, Clara Di Mario: None declared, Silvia Laura Bosello Speakers bureau: Abbvie, Pfizer, Boehringer, Laura Gigante: None declared, Alessia Musto: None declared, Gisella Vischini: None declared, Stefano Costanzi: None declared, Stefano Alivernini: None declared, Barbara Tolusso: None declared, Giuseppe Grandaliano: None declared, Elisa Gremese Speakers bureau: Abbvie, BMS, Celgene, Jannsen, Lilly, MSD, Novartis, Pfizer, Sandoz, UCB

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.

scholarly journals Henoch-Schönlein purpura outcome in children: A ten-year clinical study

2011 ◽  
Vol 139 (3-4) ◽  
pp. 174-178 ◽  
Author(s):  
Brankica Spasojevic-Dimitrijeva ◽  
Mirjana Kostic ◽  
Amira Peco-Antic ◽  
Divna Kruscic ◽  
Mirjana Cvetkovic ◽  
...  
Keyword(s):  
Renal Function ◽  
Kidney Biopsy ◽  
Renal Involvement ◽  
Immunosuppressive Treatment ◽  
Clinical Signs ◽  
Objective Evaluation ◽  
Severe Form ◽  
Renal Manifestation ◽  
Nephritic Syndrome ◽  
Nephrotic Range Proteinuria

Introduction. Henoch-Sch?nlein purpura (HSP) is the most common vasculitis of childhood. It is characterized by symptoms including nonthrombocytopenic purpura, abdominal pain, haematuria/proteinuria, and arthralgia/arthritis. The pleiomorphism of clinical signs in HSP could be confused with other conditions or other vasculitis forms. Objective. Evaluation of HSP clinical presentation, the onset and severity of renal manifestation in affected children and their outcome. Methods. A retrospective study of 49 patients diagnosed with HSP was conducted from September 1999 to September 2009. Children with severe renal manifestations (nephrotic range proteinuria, with or without nephrotic or nephritic syndrome) have undergone kidney biopsy. Results. Twenty-five patients developed renal manifestations after onset of the disease. In our study child?s older age was a risk factor for association with HSP nephritis. Six of the patients required kidney biopsy. They were successfully treated with various immunosuppressive protocols, as well as three of nine patients with nephrotic range proteinuria. Two patients developed most severe form of HSP nephritis, nephrotic-nephritic syndrome with histology grade IIIb/IVb. During the study period (average follow-up 6 years), all patients had a normal global renal function with mild proteinuria in only two cases. The prognosis of renal involvement was better than reports from other patient series. Conclusion. Long-term morbidity of HSP is predominantly attributed to renal involvement. During the study period, no patient had renal insufficiency or end stage renal disease after various combinations of immunosuppressive treatment. It is recommended that patients with HSP nephritis are followed for longer periods of time with a regular measurement of renal function and proteinuria.

P0498CLINICAL PRESENTATION AND RENAL HISTOPATHOLOGICAL FINDINGS IN PATIENTS WITH MONOCLONAL GAMMOPATHY

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Gaetano Alfano ◽  
Alice Delrio ◽  
Francesco Fontana ◽  
Annachiara Ferrari ◽  
Andrea Solazzo ◽  
...  
Keyword(s):  
Renal Function ◽  
Monoclonal Gammopathy ◽  
Kidney Biopsy ◽  
Renal Involvement ◽  
M Protein ◽  
Histological Evaluation ◽  
Al Amyloidosis ◽  
Creatinine Ratio ◽  
Urine Protein ◽  
Cast Nephropathy

Abstract Background and Aims Monoclonal gammopathy is associated with renal lesions due to the toxic effect of M-protein. The aim of our study was to evaluate the prevalence and the clinical presentation of monoclonal gammopathy in patients who underwent kidney biopsy for renal impairment. Method We conducted a retrospective study at the Nephrology and Dialysis Unit of the University Hospital of Modena from 2005 to 2017. The diagnosis of renal disease was proved histologically. Results Monoclonal gammopathy was found in 179 out of 1334 patients (13.4%). Mean age was 66.1±13.4 years with a predominance of males (63.7%). There was no differences (P=0.16) between the age of patients with benign (64.9±14.3) and malignant lymphoproliferative diseases (67.6±12). The hematologic disorders involved in the production of M-protein were MGUS (51.9%), myeloma multiple (MM) (25.7%), amyloidosis (8.9%) smoldering MM (5 %), non-Hodgkin lymphoma (NHL) (6.7%) and HL (1.7%). The prevalence of MGUS was estimated to be 6.97% (93/1334). Mean serum creatinine was 2.68±2.12 mg/dl (eGFR of 35.24±29.32 ml/min) and urine protein/creatinine ratio of 5.1±6.5. None of the study subjects progressed to MM or other lymphoproliferative diseases. The most common kidney disease was membranoproliferative (GN) (19.3%). MGRS has been identified in five patients (5.4%). Mean age of MM was 66.84±13 years. Serum concentration M-protein was 1.47±0.98. Among patients with AKI (89.1%), 13 patients (28.2%) required urgent hemodialysis. Histological evaluation showed cast nephropathy (71.7%), MM-associated AL amyloidosis (15.2%), MM-associated-light chain deposition disease (4.3%) and interstitial nephritis (8.7%). Nine patients had a diagnosis of smoldering MM. Average age was 69.17±10.8 years old. At presentation, creatinine was 2.31±2.6 mg/dl (GFR of 41.35 ml/min). Evaluation of renal biopsies allowed us to identify different patterns of glomerular diseases, expression of an aspecific renal involvement of this hematological disease. AL amyloidosis was secondary to MGUS (75%) and smoldering MM (33%). Mean age was 66.04±11.7 years old. At presentation mean urine protein/creatinine ratio was 8.33±3.2 concomitant to a serum albumin level of 2.74±0.84 gr/dl. Mean creatinine was 1.4 mg/dl corresponding to eGFR= 56.5 ml/min. Average age of NHL patients was 72.6±9.6 years. Renal function was extremely variable at presentation; mean creatinine was 2.4±1.6 mg/dl (eGFR of 30.4±22.7 ml/min). Histological evaluation of biopsy specimen revealed amyloidosis (25%), cryoglobulinemic GN (25%), LCDD (16.6%), cast-nephropathy (8.3%), LCDD (8.3%) and other renal diseases (16.8%). Three patients (1.12%) had a diagnosis of HL at mean age of 69.04±5.3 years. At presentation, renal function was normal in all patients with a creatinine of 0.93±0.07mg/dl (eGFR of 62.7.3±7.4 ml/min). Urine protein/creatinine ratio was 0.3±0.2. Kidney biopsy revealed cryoglobulinemic GN (75%) and hypertensive nephrosclerosis (25%). ANOVA analysis did not found statistically significant differences in age (p=0.11) and serum concentration of M-protein (P=0.42) between groups. The differences in mean serum creatinine and mean urine protein/creatinine ratio were statistically significant between groups, (P&lt;0.0001) and (P=0.003), respectively. A post hoc Tukey test showed that proteinuria was higher in AL amyloidosis compared to MM and HL, whereas renal function was worse in MM patients compared to the others. Conclusion MGUS was the most common monoclonal gammopathy. Surprisingly, it is frequently associated with membranoproliferative glomerulonephritis. MGRS is a rare histopathological entity (5.4%). MM manifests frequently with AKI whereas AL amyloidosis with nephrotic syndrome. Renal function was extremely variable in NHL patients; on the other hand, the limited number of HL patients with renal involvement in our cohort does not allow generalization of our findings.

scholarly journals Serum Concentrations of Laminin-P1 in Thrombotic Microangiopathy

2000 ◽  
Vol 11 (3) ◽  
pp. 434-443
Author(s):  
ALFONS SEGARRA ◽  
RAFAEL SIMÓ ◽  
LLUIS MASMIQUEL ◽  
ROSA M. SEGURA ◽  
VICENS FONOLLOSA ◽  
...  
Keyword(s):  
Renal Failure ◽  
Renal Function ◽  
Basement Membrane ◽  
Renal Involvement ◽  
Serum Levels ◽  
Renal Outcome ◽  
Serum Concentrations ◽  
Healthy Control ◽  
Regular Hemodialysis

Abstract. Laminin is the main noncollagenous constituent of the basement membrane, and its serum levels could reflect the metabolic changes that occur in the basement membrane. Severe endothelial injury with thickening of basement membrane is a characteristic feature of thrombotic microangiopathy (TMA). With this background, the aim of the study was to investigate in a prospective way (1) the relationship among serum Lam-P1, the extent of renal histopathologic lesions, and the biochemical parameters commonly used as markers of TMA activity, and (2) the usefulness of serum Lam-P1 concentrations as a renal outcome prognostic index. To this end, 18 consecutive patients with active biopsy-proven TMA with renal involvement were studied. One hundred and twenty-one healthy control subjects, 20 patients with systemic scleroderma without renal involvement, and 35 patients with systemic lupus erythematosus (20 without nephropathy and 15 with diffuse proliferative type 4 lupus nephritis) were used as control groups. In addition, to analyze the influence of either renal failure or hemodialysis therapy on serum Lam-P1 levels, 91 patients on regular hemodialysis therapy and 81 patients with predialysis chronic renal failure of different etiologies were included in the study. Serum Lam-P1 was determined by RIA at admission, on days 10 and 30 of follow-up in all patients, and after 6 and 12 mo of follow-up in all surviving patients. Serum lactate dehydrogenase, haptoglobin, platelet count, hemoglobin, and serum creatinine were determined as markers of endothelial dysfunction and hemolysis. At admission, serum levels of Lam-P1 were significantly higher in patients with TMA than in healthy control subjects (3.39 ± 0.56 U/ml versus 1.40 ± 0.18 U/ml; P < 0.0001). In addition, patients with TMA had significantly higher serum Lam-P1 levels than the other groups included in the study. At the first control, Lam-P1 correlated with lactate dehydrogenase (P = 0.006) and hemoglobin (P = 0.002). During follow-up, platelet count and hemolysis indicators normalized in all patients, while serum Lam-P1 decreased only in patients with renal function recovery. In multivariate analysis, serum creatinine and Lam-P1 at day 10 were the only independent predictors of renal outcome (r2 = 0.94; P < 0.0001) and also correlated with indices of histopathologic damage (P < 0.001). Serum Lam-P1 normalized in all patients with chronic renal failure in the samples obtained at 6 and 12 mo of regular hemodialysis after solving active TMA, thus suggesting that histopathologic lesions, but not renal function itself, would be mainly responsible for the high Lam-P1 serum concentrations detected in TMA. In conclusion, serum Lam-P1 concentrations are increased in patients with active TMA. Furthermore, patients with poor renal outcome show a prolonged increase of serum Lam-P1 that is related to the extent of renal histologic lesions. Unlike the biochemical markers of hemolysis commonly used to assess TMA activity, the sequential determination of serum Lam-P1 provides valuable information about long-term renal prognosis in patients with TMA.

FC 039RENAL OUTCOME AFTER RITUXIMAB IN ADULT-ONSET IGA VASCULITIS AND CRESCENTIC IGA NEPHROPATHY: A MULTICENTRE STUDY

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Giorgio Trivioli ◽  
Alice Canzian ◽  
Federica Maritati ◽  
Roberta Fenoglio ◽  
Evangeline Pillebout ◽  
...  
Keyword(s):  
Renal Function ◽  
Renal Disease ◽  
Iga Nephropathy ◽  
Immunosuppressive Agents ◽  
Renal Involvement ◽  
Renal Outcome ◽  
Iga Vasculitis ◽  
Adult Onset ◽  
Free Survival

Abstract Background and Aims Glucocorticoids (GC) and/or immunosuppressive agents are the mainstay of therapy for adult-onset IgA Vasculitis (IgAV), but their efficacy is often partial while their toxicity is relevant. Recently, rituximab (RTX) has been reported as a safe and effective option but only few data on renal outcome are available.1 RTX has also been used in a few cases of crescentic IgA Nephropathy (cIgAN), an IgAN subset with vasculitic lesions and poor response to conventional immunosuppressive regimens.2 We present the results of a multicentre cohort of patients with IgAV and cIgAN treated with RTX. Method The databases of 16 consorted European centres were investigated to screen for patients with adult-onset, biopsy-proven IgAV and cIgAN (crescents in ≥25% glomeruli and rapid eGFR worsening at presentation), who received RTX as induction therapy. We selected patients with active renal manifestations at the time of RTX. Remission was defined as a Birmingham Vasculitis Activity Score (BVAS)=0 or &lt;5 if it was due to persistent proteinuria and relapse as an increase in BVAS requiring change in immunosuppressive therapy. Results We identified 38 patients with IgAV and 12 patients with cIgAN who received RTX and had active renal involvement at the time of treatment. The median age at onset was 40 years (interquartile range, IQR, 25-53) and more than two-thirds of patients were male (Table 1). The median follow-up after RTX was 41 months (IQR 18-60). Renal outcomes are reported in Table 2. At the time of treatment, 24 patients (48%) had eGFR ≥60 mL/min/1.73 m2. All had IgAV and their median BVAS was 17 (IQR 10-22). Furthermore, all had microhaematuria and proteinuria. Renal histology showed mesangial or focal endocapillary proliferation in 12/17 (71%) patients who underwent biopsy (class II-IIIA according to Pillebout3). Twenty patients (83%) achieved remission; after a median of 12 months (range 9-14), four experienced a minor relapse and one had a major relapse with significant renal disease progression. Renal function remained stable in all but two patients who developed end-stage renal disease (ESRD). Micro-haematuria subsided in 14/24 (58%) and median 24h proteinuria decreased from 1750 mg (IQR 865-3275) to 175 mg (IQR 100-800) at last follow-up (p=0.029). Of the 26 patients with eGFR &lt;60 mL/min/1.73 m2, 14 had IgAV and 12 had cIgAN. All were biopsied and 20 (77%) had diffuse endo/extra-capillary proliferation (classes IIIB-IV). Five patients required dialysis but recovered soon after treatment start. Remission was achieved by 16/26 (61%); eight (50%) subsequently relapsed and two (12%) reached ESRD. At last follow-up, eGFR was ≥60 mL/min/1.73 m2 in 8/26 (31%), 10/26 (48%) had stable renal function as compared to the time of RTX, while 8/26 (31%) had developed ESRD. Median 24h proteinuria decreased from 3400 mg (IQR 2150-6500) to 770 mg (177-1315) (p=0.016). Remission rate and ESRD-free survival were respectively 86% and 92% in patients with IgAV, while they were respectively 42% and 42% in cIgAN patients. Furthermore, 21/24 (87%) patients who received RTX alone or combined to glucocorticoids but not to immunosuppressive agents achieved remission and 22/24 (92%) were ESRD-free at last follow-up. Of the 26 patients receiving immunosuppressive agents, 17 (65%) obtained remission and 18 (69%) were ESRD-free at last assessment. Over the whole follow-up, only one patient reported a severe adverse effect related to RTX (pneumonia). Conclusion Renal involvement in adult-onset IgAV and cIgAN is frequently severe. RTX, combined or not with other immunosuppressive agents, may improve renal manifestations and is well tolerated. IgAV patients show higher remission rates and a longer ESRD-free survival as compared to cIgAN patients.

scholarly journals Case Report: Pediatric Renal Sarcoidosis and Prognostic Factors in Reviewed Cases

2021 ◽  
Vol 9 ◽  
Author(s):  
Richard Klaus ◽  
Annette Friederike Jansson ◽  
Matthias Griese ◽  
Tomas Seeman ◽  
Kerstin Amann ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Case Report ◽  
Literature Review ◽  
Interstitial Nephritis ◽  
Kidney Biopsy ◽  
Renal Involvement ◽  
Oral Prednisolone ◽  
Renal Outcome ◽  
Granulomatous Interstitial Nephritis

Background: Pediatric sarcoidosis is a complex inflammatory disorder with multisystemic manifestations. Kidney involvement in children is rare, and prognostic factors are unknown.Case Report and Methods: We report the case of a 16-year-old girl with multiorgan sarcoidosis and renal involvement. The patient presented with tubulointerstitial nephritis, acute kidney injury (AKI), chest CT disseminated noduli, granulomatous iridocyclitis, giant-cell sialadenitis, and arthralgia. The kidney biopsy revealed non-granulomatous interstitial nephritis. Treatment consisted of initial high-dose methylprednisolone pulse followed by oral prednisolone and methotrexate. Full remission was achieved. In addition, we performed a literature review using PubMed and analyzed data on pediatric renal sarcoidosis cases.Results: We identified 36 cases of pediatric sarcoidosis with renal involvement on presentation and data on the end-of-follow-up glomerular filtration rate (GFR). The data from the literature review showed that renal involvement was slightly more prevalent in males (60%). AKI was present in most of the described patients (84%). Oral prednisolone was used in 35 of 36 cases; in more severe cases, other immunosuppressants were used. We newly identified renal concentration impairment and granulomatous interstitial nephritis as factors with a clear trend toward GFR loss at the end of follow-up, emphasizing the importance of kidney biopsy in symptomatic patients. In contrast, higher GFR at presentation and hypercalcemia were rather favorable factors. According to the identified predictive factors, our patient has a good prognosis and is in remission.Conclusion: The factors indicating a trend toward an unfavorable renal outcome in pediatric sarcoidosis are renal concentration impairment and granulomatous interstitial nephritis at presentation, while a higher GFR is beneficial.

Correlation Between Serological Makers and Immunofluorescence Deposits i n Kidney Tissue of Patients with Lupus Nephritis

2019 ◽  
Vol 9 (02) ◽  
Author(s):  
Haider S Al-Hadad ◽  
Aqeel Abbas Matrood ◽  
Maha Abdalrasool Almukhtar ◽  
Haider Jabur Kehiosh ◽  
Riyadh Muhi Al-Saegh
Keyword(s):  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Kidney Biopsy ◽  
Pearson Correlation ◽  
Standard Procedure ◽  
Kidney Tissue ◽  
P Value ◽  
American College Of Rheumatology ◽  
Immune Deposits ◽  
Number Of Patients

Background: Systemic lupus erythematosus (SLE) is an autoimmune disease. Few biomarkers for SLE have been validated and widely accepted for the laboratory follow-up of inflammatory activity. In SLE patients, with lupus nephritis (LN), complement activation leads to fluctuation of serum C3 and C4 that are frequently used as clinicalm biomarker of disease activity in SLE. Patients and Methods: In this study the number of patients were 37, seven patients were excluded for incomplete data collection, 28 were females ,2 were males. The duration of the study is two years from 2015 to 2017. Patients were considered to have SLE and LN according to American College of Rheumatology (ACR) criteria, and International Society of Nephrology/ Renal Pathology Society (ISN/RPS). All patients were evaluated withm clinical presentation, laboratory investigations. Our patients underwent kidney biopsy according to standard procedure by Kerstin Amann, and their tissue specimens were studied in the laboratory with light microscope (LM) and immunofluorescence microscope reagents. The relationship between the serological markers and immunofluorescence deposits in kidney biopsy of all patients were studied using the statistical analysis of Pearson correlation and single table student's T test. A P value 0.05 was considered statistically significant. Results: The granular pattern of IF deposits was present in all LN patients, and in more than two third of patients these IF deposits presented in glomerular, tubular, and mesangium sites. While less than one third of patients had IF deposits in the mesangium only. There was no statistically significant correlation between serum ANA, anti-dsDNA, and IF deposits of different types. There was significant correlation between serum C3 and C4 hypocomplementemia and IgG immune deposits in kidney biopsy, and there was significant relationship between serum C3 hypocomplementemia and full house immunofluorescence (FHIF) deposits inm kidney biopsy.Conclusions:Immunofluorescence deposits is mainly granular pattern in LN patients. There was no significant association between serum ANA, anti-dsDNA, and immune deposits in kidney tissue. Immunofluorescence deposits of IgG type correlates significantly with serum C3 and C4 hypocomplemetemia, and these immune deposits in association with low complement levels correlates with LN flare. There was significant correlation between C3 hypocomplementemia and FHIF.

scholarly journals AB0750 FUNCTIONAL CONDITION OF KIDNEYS IN THE LONG-TERM COURSE OF SLE IN ADOLESCENTS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1403.2-1403
Author(s):  
L. Bohmat ◽  
N. Shevchenko ◽  
I. Bessonova
Keyword(s):  
Renal Function ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Clinical Analysis ◽  
Average Dose ◽  
One Year

Background:Lupus nephritis is the most severe and adverse systemic lupus erythematosus (SLE) syndrome. According to modern recommendations, the clinical manifestations of active nephritis should be taken under medical control in 6 months after the start of the disease’s treatment1.Objectives:The aim of this study was to examine the functional status of the kidneys in children with SLE in the course of the disease for more than one year.Methods:The analysis included case histories of 43 patients with SLE, mostly females (41), aged 7 to 18 years (mean age 14.4 years) with disease duration of 4.75 ± 0.58 years of whom 22 were less than three years, 21 - more than three years. All children received corticosteroid therapy, at the time of the examination the average dose was 13.85 ± 1.86 mg per day in terms of prednisolone. The second component of therapy was azathioprine (average dose 97.61 ± 2.11 mg). All children received hydroxychloroquine (5 mg/kg per day).To determine the functional state of the kidneys a clinical analysis of urine, a study of the scope of specific gravity of urine during the day (Zymnytsky test), the content of creatinine and urea in serum to determine the glomerular filtration rate (GFR), the level of microalbuminuria per day were evaluated.Results:Renal involvement in the developed SLE occurred in 73.08% of patients. Among them, therapy during the first 6 months was considered quite effective in 58.06% of patients. It was found that in children with disease duration from one to three years proteinuria was registered in 68.18%, a decrease in GFR in 4.45% and hyperfiltration in 9.09%. In the group of children with duration of SLE more than three years revealed deeper changes in renal function; there was proteinuria in 90.47%, the frequency of GFR decreased was in 19.04%, a decrease of renal concentration function was in 14.28% of cases.Indicators of renal function in children with SLE depending on the duration of the disease (M ± m)IndicatorDuration of the diseasefrom 1 year to 3 years n = 22over 3 yearsn = 21Creatinine, mmol/l0,080 ± 0,0140,090 ± 0,018Мочевина, mmol/l5,66 ± 1,425,63 ± 1,61GFR, ml/min117,05 ± 19,68100,20 ± 18,98 *Microalbuminuria, mg/day24,41 ± 13,1334,73 ± 24,76Density min1,007 ± 0,0051,006 ± 0,005Density max1,024 ± 0,0051,019 ± 0,005 ***р<0,03;**р<0,01 the probability of differences when comparing between groupsConclusion:Long-term follow-up of children with SLE over one year reveals a prolongation of renal dysfunction, which worsens after three years, and is the basis for the development of irreversible renal impairment.References:[1]European evidence-based recommendations for the diagnosis and treatment of childhood-onset lupus nephritis: the SHARE initiative /Noortje Groot, Nienke de Graeff, Stephen D Marks et all. //Ann Rheum Dis. 2017 Dec;76(12):1965-1973.Disclosure of Interests:None declared

scholarly journals Proteinuria Indicates Decreased Normal Glomeruli in ANCA-Associated Glomerulonephritis Independent of Systemic Disease Activity

2021 ◽  
Vol 10 (7) ◽  
pp. 1538
Author(s):  
Désirée Tampe ◽  
Peter Korsten ◽  
Philipp Ströbel ◽  
Samy Hakroush ◽  
Björn Tampe
Keyword(s):  
Disease Activity ◽  
Systemic Disease ◽  
Renal Involvement ◽  
Kidney Injury ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Clinicopathological Characteristics ◽  
Renal Outcome ◽  
Study Results ◽  
Renal Biopsies ◽  
Stage Renal Disease

Background: Renal involvement is a common and severe complication of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), potentially resulting in a pauci-immune necrotizing and crescentic ANCA glomerulonephritis (GN) with acute kidney injury (AKI), end-stage renal disease (ESRD) or death. There is recent evidence that the degree of proteinuria at diagnosis is associated with long-term renal outcome in ANCA GN. Therefore, we here aimed to systematically describe the association between proteinuria and clinicopathological characteristics in 53 renal biopsies with ANCA GN and corresponding urinary samples at admission. Methods: A total number of 53 urinary samples at admission and corresponding renal biopsies with confirmed renal involvement of AAV were retrospectively included from 2015 to 2021 in a single-center study. Results: Proteinuria correlated with myeloperoxidase (MPO) subtype, diagnosis of microscopic polyangiitis (MPA) and severe deterioration of kidney function. Proteinuria was most prominent in sclerotic class ANCA GN and ANCA renal risk score (ARRS) high risk attributed to nonselective proteinuria, including both glomerular and tubular proteinuria. Finally, there was no association between proteinuria and systemic disease activity, suggesting that proteinuria reflected specific renal involvement in AAV rather that systemic disease activity. Conclusions: In conclusion, proteinuria correlated with distinct clinicopathological characteristics in ANCA GN, mostly attributed to a reduced fraction of normal glomeruli. Furthermore, proteinuria in ANCA GN reflected specific renal involvement in AAV rather than systemic disease activity. Therefore, urinary findings could further improve our understanding of mechanisms promoting kidney injury and progression of ANCA GN.

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