scholarly journals The Clinical and Pathological Features of Children With Microscopic Polyangiitis

2021 ◽  
Vol 9 ◽  
Author(s):  
Qian Li ◽  
Li-Chun Yu ◽  
Feng-Xia Li ◽  
Jing Wang ◽  
Yuan Chen ◽  
...  
Keyword(s):  
Microscopic Polyangiitis ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Antithyroid Drug ◽  
Good Prognosis ◽  
Factors Associated ◽  
Stage Renal Disease ◽  
End Stage ◽  
Lung Impairment

Objective: The aim of this study was to explore the clinical features, pathological characteristics, and the prognosis of children with microscopic polyangiitis (MPA).Methods: Ten children with MPA that were hospitalized in our hospital were included in this study. The children's pre-diagnosis status, clinical manifestations, renal pathology, treatment, and prognosis data were analyzed retrospectively.Results: All 10 cases included female patients with a median age of 8.9 years old at the time of diagnosis. MPO-ANCA antibody was positive in all cases, combined with a positive anti-GBM antibody in two cases. Nine cases had primary AAV and one had antithyroid drug (ATD)-associated MPA (secondary to methimazole). Renal involvement was found in all 10 patients, lung impairment was present in eight cases, and anemia was present in nine patients. Renal biopsies were performed in all 10 patients. Segmental focal or global glomerular necrosis was observed in 70% of the patients (7/10). The treatment mainly included steroid use combined with Cyclophosphamide and Mycophenolate. The follow-up s of the patients revealed normal renal function in eight patients and progression to end-stage renal disease (ESRD) in two patients.Conclusions: Female predisposition and positive MPO-ANCA antibody were prominent in children with MPA. The patients' kidneys and lungs were the most frequently involved organs. Corticosteroid combined with immunosuppressive therapy was recommended for the treatment of MPA. Early diagnosis, prompt aggressive treatment, and regular follow-ups are also very important factors associated with a good prognosis.

MO282EARLY RENAL RECOVERY AFTER A FIRST FLARE OF PAUCI IMMUNE GLOMERULONEPHRITIS

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.

scholarly journals Adverse outcomes associated with rapid linear and non-linear patterns of chronic kidney disease progression

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Ibrahim Ali ◽  
Rajkumar Chinnadurai ◽  
Sara T. Ibrahim ◽  
Philip A. Kalra
Keyword(s):  
Adverse Outcomes ◽  
Individual Risk ◽  
Rapid Progression ◽  
Study Cohort ◽  
Factors Associated ◽  
Non Linear ◽  
Stage Renal Disease ◽  
End Stage ◽  
Egfr Slope

Abstract Background Patients with rapidly declining renal function face the dual threat of end-stage renal disease (ESRD) and mortality prior to ESRD. What is less well characterised is whether the pattern of the renal trajectory, linear or non-linear, unmasks subgroups of rapidly progressing patients that face adverse outcomes in a differential manner. Methods An individual eGFR slope was applied to all outpatient estimated glomerular filtration rate (eGFR) values for each patient in the Salford Kidney Study from 2002 to 2018 who had at least 2 years follow-up, ≥4 eGFR values and baseline eGFR 15 to < 60 ml/min/1.73m2. Rapid progression was defined as an annual eGFR slope of ≤ − 3 ml/min/1.73m2/yr and patients were categorised as linear or non-linear progressors based on the nature of their eGFR-time graphs. A Fine-Gray competing risk hazard model was used to determine factors associated with progression to ESRD and with mortality prior to ESRD. Cumulative incidence function curves highlighted differences in outcomes between linear and non-linear patients. Results There were 211 rapidly deteriorating patients with linear eGFR trajectories and 61 rapid non-linear patients in the study cohort. Factors associated with ESRD included younger age, male gender, lower baseline eGFR and higher serum phosphate, whilst older age, history of myocardial infarction and anaemia predicted mortality prior to ESRD. Over a median follow-up of 3.7 years, linear progressors reached ESRD sooner whilst those with non-linear progression faced significantly higher rates of mortality prior to ESRD. Conclusions Patients with rapid eGFR decline have high rates of adverse outcomes that are differentially expressed in those progressing linearly and non-linearly as a result of differing phenotypic profiles. Consequently, addressing individual risk factor profiles is important to deliver optimal personalised patient care.

scholarly journals Fibrosis-5 predicts end-stage renal disease in patients with microscopic polyangiitis and granulomatosis with polyangiitis without substantial liver diseases

2021 ◽  
Author(s):  
Hyeok Chan Kwon ◽  
Jason Jungsik Song ◽  
Yong-Beom Park ◽  
Sang-Won Lee
Keyword(s):  
Renal Disease ◽  
End Stage Renal Disease ◽  
Liver Diseases ◽  
Microscopic Polyangiitis ◽  
Laboratory Data ◽  
All Cause Mortality ◽  
Poor Outcomes ◽  
Stage Renal Disease ◽  
End Stage

AbstractWe previously reported that fibrosis-4 (FIB-4) was associated with poor outcomes of microscopic polyangiitis (MPA) and granuloma with polyangiitis (GPA). We also investigated the potential of FIB-5, a novel index, in predicting all-cause mortality and end-stage renal disease (ESRD) during follow-up in patients with MPA and GPA without substantial liver diseases. Clinical and laboratory data at diagnosis were collected by reviewing the medical records of 180 patients with MPA and GPA. FIB-5 was obtained by a following equation: FIB-5 = (serum albumin (g/L) × 0.3 + platelet count (109/L) × 0.05) − (alkaline phosphatase (IU/L) × 0.014 + aspartate aminotransferase/alanine aminotransferase ratio × 6 + 14). The median age of the patients at diagnosis was 61.0 years. FIB-5 at diagnosis could not reflect the cross-sectional vasculitis activity. The cutoffs of FIB-5 for poor outcomes was set as 0.82 (the lowest tertile) and -0.42 (the lowest quartile) at diagnosis. In Kaplan–Meier survival analysis, patients with FIB-5 < 0.82 and those with FIB-5 < -0.42 exhibited lower ESRD-free survival rates than those without. However, it could not predict all-cause mortality. In multivariable Cox hazards analysis, both FFS (Hazard ratio (HR) 1.554) and FIB-5 < 0.82 (HR 2.096) as well as both FFS (HR 1.534) and FIB-5 < -0.42 (HR 2.073) at diagnosis independently predicted ESRD during follow-up. In conclusion, FIB-5 < 0.82 and FIB-5 < -0.42 at diagnosis could predict the occurrence of ESRD, but not all-cause mortality, during follow-up in patients with MPA and GPA without substantial liver diseases.

scholarly journals FRAILTY AND HEALTH-RELATED QUALITY OF LIFE IN END STAGE RENAL DISEASE PATIENTS OF ALL AGES

2016 ◽  
pp. 1-6
Author(s):  
M.A. MCADAMS-DEMARCO ◽  
H. YING ◽  
I. OLORUNDARE ◽  
E.A. KING ◽  
N. DESAI ◽  
...  
Keyword(s):  
Initial Assessment ◽  
Poor Hrqol ◽  
Factors Associated ◽  
Related Quality ◽  
Health Related ◽  
Fair Poor ◽  
Stage Renal Disease ◽  
End Stage

Background: Frailty is associated with worse health-related quality of life (HRQOL) in older adults and worse clinical outcomes in adults of all ages with end stage renal disease (ESRD). It is unclear whether frail adults of all ages with ESRD are more likely to experience worse HRQOL. Objective: The goal of this study was to identify factors associated with worsening HRQOL in this population. Design, setting and measurements: We studied 233 adults of all ages with ESRD enrolled (11/2009-11/2013) in a longitudinal cohort study. Frailty status was measured at enrollment and HRQOL was reported (Excellent, Very Good, Good, Fair or Poor) at the initial assessment and follow-up (median follow-up 9.4 months). We studied factors associated with Fair/Poor HRQOL at follow-up using logistic regression and factors associated with HRQOL change using multinomial regression. All models were adjusted for age, sex, race, education, BMI, diabetes status, history of a previous transplant, type of dialysis and time between assessments. Results: Fair/Poor HRQOL was reported by 28% at initial assessment and 33% at follow-up. 47.2% of participants had stable HRQOL, 22.8% better HRQOL, and 30.0% worse HRQOL at follow-up (P<0.001). In adjusted models, only frailty was associated with Fair/Poor HRQOL at follow-up (OR: 2.79, 95% CI: 1.32-5.90) and worsening HRQOL at follow-up (RR: 2.91, 95%CI: 1.08-7.80). Conclusions: Frail adults of all ages with ESRD are more likely to experience fair/poor HRQOL and worsening HRQOL over time. Frailty represents a state of decreased physiologic reserve that impacts not only clinical outcomes but also the patient-centered outcome of HRQOL.

scholarly journals P1432THE PRE-DIALYSIS CARE TRAJECTORIES OF ESRD PATIENTS: WHAT TYPES AND WHAT FACTORS OF EMERGENCY DIALYSIS START?

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Maxime Raffray ◽  
Adélaïde Pladys ◽  
Fei Gao ◽  
Cécile Couchoud ◽  
Cécile Vigneau ◽  
...  
Keyword(s):  
Multiple Correspondence Analysis ◽  
Healthcare Consumption ◽  
Logistic Regression Models ◽  
Factors Associated ◽  
Qualitative Approaches ◽  
Stage Renal Disease ◽  
End Stage ◽  
Esrd Patients ◽  
Rein Registry

Abstract Background and Aims According to the REIN registry, in France in 2017, 30% of incident end-stage renal disease (ESRD) patients started dialysis in emergency. Emergency dialysis start (ES) is associated with higher risks of morbidity and mortality as well as a decreased quality of life for patients. The objectives were to describe groups of patients with similar care trajectories (or “types”) and identify the factors associated with ES. Method Data from the French REIN registry linked with the French national health insurance database (SNDS) were used. Patients aged 18 years old and older who started dialysis in 2015 in France were included. The 2 years pre-dialysis care trajectory was defined using 8 key components of ambulatory and hospital CKD care consumption. Multiple Correspondence Analysis (MCA) on these components was performed before a Hierarchical Clustering on Principal Components (HCPC) in order to identify types of care trajectories. Logistic regression models including both patients’ characteristics and the key CKD healthcare consumption were used to identify factors associated with ES. Results Among the 8856 patients included, 2681 (30.3%) started dialysis in emergency. The HCPC identified 6 types of pre-dialysis care trajectories. ES rate ranged from 11% to 62% depending on the type. Patient’s profiles were also different between types of trajectories, including age and comorbidities (diabetes prevalence ranged from 22% to 56%). For example, the 5th and 6th types of pre-dialysis care trajectories with high ES rates were characterized by low and absent CKD healthcare consumption respectively. They were shared by younger and less comorbid patients (26.7% and 25.7% aged &lt; 45 years old, 66.5% and 59.4% without any cardiovascular diseases respectively). Conversely to the absence of nephrologist follow-up, the absence of general practitioner follow-up was not significantly associated with ES (OR=1.83, CI 95% [1.58-2.13] and OR=0.79, CI 95% [0.61-1.04] respectively). Conclusion Exploiting the strength of the combined use of clinical and healthcare consumption data, our study shows the diversity of pre-dialysis care trajectories of CKD patients. While some of these types of trajectories can be explained by patients’ clinical and demographic characteristics, complementary qualitative approaches are required to fully understand ES. Some types, as well as ES associated factors, highlight the need for earlier CKD screening and nephrologist referral.

scholarly journals Prognostic factors for chronic kidney disease and end-stage renal disease in patients with lupus nephritis: A retrospective cohort study

2021 ◽  
Vol 5 (1) ◽  
pp. 034-041
Author(s):  
Ferreira Braga Fernanda Nogueira Holanda ◽  
das Chagas Medeiros Marta Maria ◽  
Viana Jr. Antônio Brazil ◽  
Maia Barros Levi Coelho ◽  
Pontes Marcelo Ximenes ◽  
...  
Keyword(s):  
Chronic Kidney Disease ◽  
Cohort Study ◽  
Prognostic Factors ◽  
End Stage Renal Disease ◽  
Retrospective Cohort ◽  
Tertiary Hospital ◽  
Factors Associated ◽  
Stage Renal Disease ◽  
End Stage

Background: Lupus Nephritis (LN) occurs in approximately half of all patients with Systemic Lupus Erythematosus (SLE) and it is the most common cause of morbidity and mortality in patients with SLE. Factors associated with poor renal outcome vary among studies, and researches coming from Brazil are scarce. Objectives: To identify the prognostic factors associated to the development of Chronic Kidney Disease (CKD) and End Stage Renal Disease (ESRD) in LN patients followed in a tertiary hospital. Design and Settings: We conducted a retrospective cohort study set in a tertiary hospital in Fortaleza, Ceará, Brazil. Methods: We compiled a total of 214 LN patients diagnosed between 1983 and 2015. Data was collected from medical records and further analyzed using logistic regression. Results: LN prevalence was 53.9%. The cohort had a mean follow-up of 11.2 years (SD ± 7.2 years). At the end of follow-up, 93 of 197 patients (47.2%) had CKD, and 49 of 191 (25.6%) were on regular dialysis. The main factors associated for developing CKD after logistic regression analysis were the following predictors: hypertension (HR 2.80; 95% CI 1.30-6.01; p = 0.008), time between diagnosis of SLE and diagnosis of LN (HR 0.98; 95% CI 0.97-0.99; p = 0.009) and discontinuation of medications (HR 2.41; 95% CI 1.08-5.37; p = 0.03). Conclusion: Hypertension, discontinuation of medications, and time between diagnosis of SLE and diagnosis of LN are independent variables associated with the development of CKD and ESDR in our study.

scholarly journals Determinants of mortality in elderly patients with tuberculosis: a population-based follow-up study

2017 ◽  
Vol 145 (7) ◽  
pp. 1374-1381 ◽  
Author(s):  
Y.-F. YEN ◽  
J.-Y. FENG ◽  
S.-W. PAN ◽  
P.-H. CHUANG ◽  
V. Y.-F. SU ◽  
...  
Keyword(s):  
Elderly Patients ◽  
Chest Radiograph ◽  
Economic Status ◽  
Population Based ◽  
Directly Observed Therapy ◽  
Factors Associated ◽  
Control Programmes ◽  
Stage Renal Disease ◽  
End Stage

SUMMARYElderly individuals with tuberculosis (TB) are more likely to have a non-specific clinical presentation of TB and high mortality. However, factors associated with mortality in elderly TB patients have not been extensively studied. This retrospective cohort study aimed to identify factors associated with death among elderly Taiwanese with TB. All elderly patients with TB from 2006 to 2014 in Taipei, Taiwan, were included in a study. Multiple logistic regression was used to identify the factors associated with death in elderly TB patients. The mean age of the 5011 patients was 79·7 years; 74·1% were men; 32·7% had mortality during the study follow-up period. After controlling for potential confounders, age ⩾75 years (reference: 65–74 years), male sex, end-stage renal disease (ESRD), malignancy, acid-fast bacilli-smear positivity, TB-culture positivity, pleural effusion on chest radiograph and notification by an ordinary ward or intensive care unit were associated with a higher risk of all-cause death; while high school, and university or higher education, cavity on chest radiograph and directly observed therapy were associated with a lower risk of all-cause death. This study found that the proportion of death among elderly patients with TB in Taipei, Taiwan, was high. To improve TB treatment outcomes, future control programmes should particularly target individuals with comorbidities (e.g. ESRD and malignancy) and those with a lower socio-economic status (e.g. not educated).

scholarly journals Endovascular mechanical thrombectomy and stenting in a case of central vein thrombosis

2021 ◽  
Vol 14 (2) ◽  
pp. e236508
Author(s):  
Rajesh Vijayvergiya ◽  
Navjyot Kaur ◽  
Saroj K Sahoo ◽  
Ashish Sharma
Keyword(s):  
Mechanical Thrombectomy ◽  
Transluminal Angioplasty ◽  
Brachiocephalic Vein ◽  
Central Vein ◽  
Vein Thrombosis ◽  
Catheter Directed Thrombolysis ◽  
Stage Renal Disease ◽  
End Stage ◽  
Vein Stenosis

Central vein stenosis and thrombosis are frequent in patients on haemodialysis for end-stage renal disease. Its management includes anticoagulation, systemic or catheter-directed thrombolysis, mechanical thrombectomy and percutaneous transluminal angioplasty (PTA). Use of mechanical thrombectomy in central vein thrombosis has been scarcely reported. We hereby report a case of right brachiocephalic vein thrombosis with underlying stenosis, which was successfully treated by mechanical thrombectomy followed by PTA and stenting. The patient had a favourable 10 months of follow-up.

scholarly journals AB0820 INFLUENCE OF ANCA ANTIBODIES ON DEMOGRAPHIC AND CLINICAL CHARACTERISTIC OF AAV

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1434.1-1434
Author(s):  
K. Wójcik ◽  
A. Masiak ◽  
Z. Zdrojewski ◽  
R. Jeleniewicz ◽  
M. Majdan ◽  
...  
Keyword(s):  
Renal Disease ◽  
End Stage Renal Disease ◽  
Clinical Characteristics ◽  
Renal Involvement ◽  
Organ Involvement ◽  
Unknown Etiology ◽  
Anca Associated Vasculitis ◽  
Stage Renal Disease ◽  
End Stage ◽  
Male To Female

Background:ANCA associated vasculitides (AAV) are a heterogeneous group of rare diseases with unknown etiology and the broad clinical spectrum ranging from life-threatening systemic disease, through single organ involvement to minor isolated skin changes. Unfortunately the clinical classification, ANCA specificity or genetic characteristics alone is not able to categorize AAV patients in a satisfactory manner. As a consequence advanced statistical techniques were used to identify and stratify AAV subphenotypes [1, 2]. Here we have analyzed influence of the ANCA type on clinical manifestations and demographic characteristics in various types of AAV, based on data from the POLVAS registryObjectives:We decided to retrospectively analyze a large cohort of Polish AAV patients deriving from several referral centers – members of the Scientific Consortium of the Polish Vasculitis Registry (POLVAS) – and concentrate on demographic and clinical characteristics of anti-PR3 and anti-MPO positive patients regardless of their clinical diagnosis.Methods:We conducted a systematic multicenter retrospective study of adult patients diagnosed with AAV between Jan 1990 and Dec 2016. Patients were enrolled by 9 referral centers. We analyzed dichotomous variables: gender; ANCA status – anti-PR3+ or anti-MPO+, ANCA negative; organ involvement - skin, eye, ENT, respiratory, heart, GI, renal, urinary, CNS, peripheral nerves and polytomous variable (number of relapses), supported by quantitative covariates (e.g., age at diagnosis, CRP at diagnosis, maximal serum creatinine concentration ever)[3].Results:MPO-positive patients (both GPA and EGPA phenotype) were older at the time of diagnosis with a substantial percentage diagnosed > 65 years of age, and with high rate of renal involvement. Interestingly, while in the whole group of patients diagnosed with EGPA male to female ratio was 1:2, the MPO+ EGPA patients showed M:F ratio of 1:1.The analysis of ANCA negative AAV reveled significant differences in GPA, ANCA negative group is characterized with significantly lower frequency of renal involvement compared to rest GPA (11,5% vs 63,7%) p<0,05 what should be emphasized ANCA negative AAV never lead to ESRD (end stage renal disease) or even transient dialysis.Conclusion:ANCA specificity is indispensable as a separate variable in any clinically relevant analysis of AAV subcategories. MPO+ group is characterized by older age at time of diagnosis, male to female ration 1:1, kidney involvement, and shows more homogenous clinical phenotype than PR3+ AAV patients. In our group ANCA negative AAV never lead to ESRD (end stage renal disease) or even transient dialysis.References:[1]Mahr A, Specks U, Jayne D. Subclassifying ANCA-associated vasculitis: a unifying view of disease spectrum. Rheumatol Oxf Engl 2019;58:1707–9. https://doi.org/10.1093/rheumatology/kez148.[2]Wójcik K, Biedroń G, Wawrzycka-Adamczyk K, Bazan-Socha S, Ćmiel A, Zdrojewski Z et al. Subphenotypes of ANCA-associated vasculitis identified by latent class analysis. Clin Exp Rheumatol. 2020 Sep 1. Epub PMID: 32896241.[3]Wójcik K, Wawrzycka-Adamczyk K, Włudarczyk A, Sznajd J, Zdrojewski Z, Masiak A, et al. Clinical characteristics of Polish patients with ANCA-associated vasculitides—retrospective analysis of POLVAS registry. Clinical Rheumatology. 1 wrzesień 2019;38(9):2553–63.Disclosure of Interests:None declared

scholarly journals Living Kidney Donation: Practical Considerations on Setting Up a Program

2021 ◽  
Vol 2 (1) ◽  
pp. 75-86
Author(s):  
Maria Irene Bellini ◽  
Vito Cantisani ◽  
Augusto Lauro ◽  
Vito D’Andrea
Keyword(s):  
Good Practice ◽  
Kidney Donation ◽  
Living Kidney Donation ◽  
Exchange Programs ◽  
Paired Exchange ◽  
Stage Renal Disease ◽  
End Stage ◽  
Additional Value ◽  
Work Up

Living kidney donation represents the best treatment for end stage renal disease patients, with the potentiality to pre-emptively address kidney failure and significantly expand the organ pool. Unfortunately, there is still limited knowledge about this underutilized resource. The present review aims to describe the general principles for the establishment, organization, and oversight of a successful living kidney transplantation program, highlighting recommendation for good practice and the work up of donor selection, in view of potential short- and long-terms risks, as well as the additional value of kidney paired exchange programs. The need for donor registries is also discussed, as well as the importance of lifelong follow up.

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