Prevalence of chronic kidney disease in France: methodological considerations and pitfalls with the use of Health claims databases.

Background: Health policy-making require careful assessment of chronic kidney disease (CKD) epidemiology to develop efficient and cost-effective care strategies. The aim of the present study was to use the RENALGO-EXPERT algorithm to estimate the global prevalence of CKD in France. Methods: An expert group developed the RENALGO-EXPERT algorithm based on healthcare consumption. This algorithm has been applied to the French National Health claims database (SNDS), where no biological test findings are available to estimate a national CKD prevalence for the years 2018-2021. The CONSTANCES cohort (+219 000 adults aged 18-69 with one CKD-EPI eGFR) was used to discuss the limit of using health claims data. Results: Between 2018 and 2021, the estimated prevalence in the SNDS increased from 8.1% to 10.5%. The RENALGO-EXPERT algorithm identified 4.5% of the volunteers in the CONSTANCES as CKD. The RENALGO-EXPERT algorithm had a positive predictive value of 6.2% and negative predictive value of 99.1% to detect an eGFR<60 ml/min/1.73 m². Half of 252 false positive cases (ALGO+, eGFR > 90) had been diagnosed with kidney disease during hospitalization, and the other half based on healthcare consumption suggestive of a 'high-risk' profile; 95% of the 1661 false negatives (ALGO-, eGFR < 60) had an eGFR between 45 and 60 ml/min, half had medication and two-thirds had biological exams possibly linked to CKD. Half of them had a hospital stay during the period but none had a diagnosis of kidney disease. Conclusions: Our result is in accordance with other estimations of CKD prevalence in the general population. Analysis of diverging cases (FP and FN) suggests using health claims data have inherent limitations. Such an algorithm can identify patients whose care pathway is close to the usual and specific CKD pathways. It does not identify patients who have not been diagnosed or whose care is inappropriate or at early stage with stable GFR.

Anti-GBM antibody in a patient with diabetic nephropathy; all that glitters is not gold.

We present the case of a 58-year-old male diabetic patient admitted to our department for a slight decrease in kidney function, with nephrotic range proteinuria, hematuria (16,000/ml) and positive anti-glomerular basement membrane antibodies. Kidney biopsy revealed diabetic nephropathy with no evidence of crescent formation or linear immunoglobulin deposits along the basement membrane. We discuss the various clinical settings involving positive anti-glomerular basement membrane in the absence of crescentic glomerulonephritis.

Hot Spot of Complement Factor I Rare Variant p.Ile357Met in Patients With Hemolytic Uremic Syndrome.

Atypical hemolytic uremic syndrome (aHUS) is a rare kidney disease due to a dysregulation of the complement alternative pathway. Complement factor I (CFI) negatively regulates the alternative pathway and CFI gene rare variants have been associated to aHUS with a low disease penetrance. We report 10 unrelated cases of HUS associated to a rare CFI variant, p.Ile357Met (c.1071T>G). All patients with isolated p.Ile357Met CFI missense variant were retrospectively identified among patients included between January 2007 and January 2022 in the French HUS Registry. We identified 10 unrelated patients (70% women; median age at HUS diagnosis, 36.5 years) who carry the same rare variant p.Ile357Met in the CFI gene. Seven patients (cases 1-7) presented with aHUS in the native kidney associated with malignant hypertension in 5 patients. None received a C5 inhibitor. Two of these cases occurred in the peripartum period with complete recovery of kidney function, while 5 of these patients reached kidney failure requiring replacement therapy (KFRT). Four patients with KFRT subsequently underwent kidney transplantation. Three later developed C3 glomerulopathy in their kidney graft, but none had aHUS recurrence. Three other patients (cases 8-10) experienced de novo thrombotic microangiopathy after kidney transplantation, precipitated by various triggers. The rare CFI variant p.Ile357Met appears to be a facilitating genetic factor for HUS and for some forms of secondary HUS.

General practitioners' representation of early-stage CKD is a barrier to adequate management and patient empowerment: a phenomenological study.

BACKGROUND: In high-income countries, chronic kidney disease (CKD) affects over 10% of the population. Identifying these patients early is a priority, especially as new treatments are available to reduce the risk of cardiovascular and renal morbidity. We aimed at understanding the management and care pathway of patients with early-to-moderate CKD defined as an estimated glomerular filtration rate (eGFR) ≥ 45 mL/min/1.73 m2 (CKD-EPI), by analyzing the experience of general practitioners in a region in France. METHODS: This qualitative semiopragmatic phenomenological study analyzed in-depth interviews held with a purposive sample (age, gender, training, type of practice, rural/urban context) of 24 general practitioners, with triangulation of research until data saturation. RESULTS: From diagnostic, etiological and prognostic viewpoints, the general practitioners enrolled in our study perceived CKD as a complex, poorly-defined clinical entity in asymptomatic and multimorbid patients. They distinguished it from a rare condition they considered as 'mainly renal'. The fact that they did not perceive early-stage CKD as a disease was a hindrance to patient care, which should protect the kidneys with a preventive approach. Indeed, general practitioners perceived CKD patient management as a pathway requiring a personalized, integrative model, common to all chronic diseases, without necessarily involving a nephrologist, at least in the early stages. CONCLUSIONS: This study shows how the general practitioners' representations influence their attitudes and interventions. Clarifying the concept of early-stage CKD by taking factors like age and etiology into account would facilitate personalized management of this heterogeneous, often multimorbid, population. Finally, organizational models to support patient empowerment in an integrative care pathway must be established and validated.

Estimated Glomerular Filtration Rate, Albuminuria, and Adverse Outcomes: An Individual-Participant Data Meta-Analysis.

Importance: Chronic kidney disease (low estimated glomerular filtration rate [eGFR] or albuminuria) affects approximately 14% of adults in the US. Objective: To evaluate associations of lower eGFR based on creatinine alone, lower eGFR based on creatinine combined with cystatin C, and more severe albuminuria with adverse kidney outcomes, cardiovascular outcomes, and other health outcomes. Design, Setting, and Participants: Individual-participant data meta-analysis of 27 503 140 individuals from 114 global cohorts (eGFR based on creatinine alone) and 720 736 individuals from 20 cohorts (eGFR based on creatinine and cystatin C) and 9 067 753 individuals from 114 cohorts (albuminuria) from 1980 to 2021. Exposures: The Chronic Kidney Disease Epidemiology Collaboration 2021 equations for eGFR based on creatinine alone and eGFR based on creatinine and cystatin C; and albuminuria estimated as urine albumin to creatinine ratio (UACR). Main Outcomes and Measures: The risk of kidney failure requiring replacement therapy, all-cause mortality, cardiovascular mortality, acute kidney injury, any hospitalization, coronary heart disease, stroke, heart failure, atrial fibrillation, and peripheral artery disease. The analyses were performed within each cohort and summarized with random-effects meta-analyses. Results: Within the population using eGFR based on creatinine alone (mean age, 54 years [SD, 17 years]; 51% were women; mean follow-up time, 4.8 years [SD, 3.3 years]), the mean eGFR was 90 mL/min/1.73 m2 (SD, 22 mL/min/1.73 m2) and the median UACR was 11 mg/g (IQR, 8-16 mg/g). Within the population using eGFR based on creatinine and cystatin C (mean age, 59 years [SD, 12 years]; 53% were women; mean follow-up time, 10.8 years [SD, 4.1 years]), the mean eGFR was 88 mL/min/1.73 m2 (SD, 22 mL/min/1.73 m2) and the median UACR was 9 mg/g (IQR, 6-18 mg/g). Lower eGFR (whether based on creatinine alone or based on creatinine and cystatin C) and higher UACR were each significantly associated with higher risk for each of the 10 adverse outcomes, including those in the mildest categories of chronic kidney disease. For example, among people with a UACR less than 10 mg/g, an eGFR of 45 to 59 mL/min/1.73 m2 based on creatinine alone was associated with significantly higher hospitalization rates compared with an eGFR of 90 to 104 mL/min/1.73 m2 (adjusted hazard ratio, 1.3 [95% CI, 1.2-1.3]; 161 vs 79 events per 1000 person-years; excess absolute risk, 22 events per 1000 person-years [95% CI, 19-25 events per 1000 person-years]). Conclusions and Relevance: In this retrospective analysis of 114 cohorts, lower eGFR based on creatinine alone, lower eGFR based on creatinine and cystatin C, and more severe UACR were each associated with increased rates of 10 adverse outcomes, including adverse kidney outcomes, cardiovascular diseases, and hospitalizations.

Prevalence of anti-neutrophil cytoplasmic antibody-associated vasculitis in the south of France, using the capture-recapture method.

OBJECTIVES: This study aimed to estimate the prevalence of ANCA-associated vasculitis (AAV), ie granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), in Southern France in 2018, and evaluate differences among Europeans and non-Europeans. METHODS: This population-based, cross-sectional study used four sources (hospitals, community-based physicians, laboratories, National Health Insurance) to identify adults ≥ 15 years diagnosed with GPA, MPA or EGPA, living in Hérault and Gard in 2018. Cases were defined using the ACR/EULAR classification criteria, and if necessary, the European Medicines Agency algorithm. Prevalence estimates were standardised to the world population and capture-recapture analysis was used to assess the comprehensiveness of the estimation. The influence of geographical origin was evaluated. RESULTS: 202 patients were selected, with 86 cases of GPA (42.6%), 85 cases of MPA (42.1%), and 31 cases of EGPA (15.3%). The standardised prevalence estimates per million inhabitants for 2018 were: 103 (95%CI 84 - 125) for AAV, 48 (95%CI 35 - 64) for GPA, 39 (95%CI 28 - 53) for MPA and 16 (95%CI 9 - 26) for EGPA, 36 (95%CI 25 - 50) for anti-PR3 positive AAV, 46 (95%CI 34 - 61) for anti-MPO positive AAV, and 16 (95%CI 9 - 26) for ANCA-negative AAV. The global estimation of comprehensiveness by capture-recapture analysis was 80.5%. The number of AAV cases was higher for non-European residents (P=0.001), particularly for MPA (P<0.0001). CONCLUSION: We provide a new estimate of AAV prevalence in France and show a higher prevalence of MPA in non-European patients.

Kidney involvement in hereditary transthyretin amyloidosis: a cohort study of 103 patients.

Background: Hereditary transthyretin amyloidosis (ATTRv) is a disabling and life-threatening disease that primarily affects the nervous system and heart. Its kidney involvement has not been systematically studied, particularly in non-V30M mutations, and is not well known to nephrologists. Methods: We conducted a retrospective study describing the kidney phenotype of all prevalent patients with ATTR mutations, with neurological or cardiac involvement or presymptomatic carriers, followed up in two university hospitals from the South of France between June 2011 and June 2021. Results: A total of 103 patients were included, among whom 79 were symptomatic and 24 were presymptomatic carriers. Patients carried 21 different ATTR mutations and 54% carried the V30M mutation. After a mean follow-up of 7.9 ± 25.7 years, 30.4% of the symptomatic patients had developed chronic kidney disease (CKD) and 20.3% had a urinary protein:creatinine ratio ≥0.5 g/g. None of the presymptomatic carriers had CKD or proteinuria. In a multivariate analysis, late onset of symptoms (after 60 years), the V122I mutation and proteinuria were significantly associated with CKD. The median CKD-free survival in symptomatic patients was estimated at 81.0 years (interquartile range 77.1-84.9). It did not differ between V30M and non-V30M patients, but was lower in patients with the V122I mutation. The average age of the onset of CKD was 69.3 ± 13.0 years. In one 38-year-old V30M female who presented a kidney-predominant phenotype, treatment with patisiran resulted in remission of the nephrotic syndrome. Conclusion: CKD affects almost one-third of patients with symptomatic ATTRv. The role of ATTRv per se in the development of CKD in this population remains to be determined, but some patients may benefit from specific therapies.

Interest of a Kidney Biopsy to Rule out ANCA-Associated Renal Vasculitis in Glomerulonephritis Patients with a Positive ANCA.

Kidney biopsy is the gold standard for diagnosing glomerular kidney disease. Some authors debate the necessity of systematically performing kidney biopsies in ANCA-associated vasculitis (AAV) to confirm the diagnosis and assess the severity of renal damage. Nevertheless, kidney involvement is considered an organ-threatening disease requiring an aggressive immunosuppressive regimen. We present a series of 4 cases with a high clinical suspicion of ANCA-associated crescentic glomerulonephritis based on rising serum creatinine, presence of proteinuria and/or hematuria, and presence of ANCA with specificity against PR-3 or MPO. The main diagnosis, however, was arterionephrosclerosis without renal AAV. Certain comorbidities, such as diabetes and/or high blood pressure, can quickly mimic progressive glomerulonephritis. In addition, some patients with AAV do not have high creatinine, proteinuria, or hematuria levels. ANCA alone is not specific to AAV and has a poor positive predictive value. The main concern is to prevent the unnecessary, inappropriate complications of heavy immunosuppression, i.e., serious infections or risk of future malignancies. Kidney pathological confirmation is important in patients with no compatible extra-renal manifestations of AAV or any other possible renal diagnosis such as may be found in polyvascular disease or diabetic patients.

Specificity of severe AKI aetiology and care in the elderly. The IRACIBLE prospective cohort study.

INTRODUCTION: Acute Kidney Injury (AKI) is increasingly common in people over 65 years of age, but its causes and management are poorly described. The purpose of this study was to describe the causes, management and prognosis of patients over 65 hospitalised for severe acute kidney injury (AKI) in all departments of a tertiary centre. METHOD: The prospective IRACIBLE (IRA: AKI in French; CIBLE: target in French) cohort included 480 patients hospitalised at a university hospital over 18 months for severe AKI or subgroup of AKIN3 (Acute Kidney Injury Network classification) defined by an acute creatinine increase > 354 µmol/L or managed with acute renal replacement therapy (RRT). The history, aetiology of AKI, management, and prognosis were compared in three age groups: < 65, 65-75, and > 75 years. RESULTS: The study population included 480 subjects (73% men) with a median body mass index (BMI) of 26.6 kg/m2 [23.3, 30.9], 176 (37%) diabetic patients, 124 (26%) patients < 65 years, 150 (31%) 65-75 years and 206 (43%) > 75 years. Increasing age class was associated with more comorbidities, a significantly lower median estimated glomerular filtration rate (eGFR) 6 months before inclusion (82; 62; 46 ml/min/1.73 m2, p < 0.05) and aetiology of AKI, which was more often obstructive (12%; 15%; 23%, p = 0.03) or part of a cardio-renal syndrome (6%; 9%; /15%, p = 0.04). Older patients were less often managed in the intensive care unit  (54%; 47%; 24%, p < 0.0001), were less frequently treated by RRT (52%; 43%; 31%, p < 0.001) and received fewer invasive treatments  (6%; 9%; 22%, p < 0.0001). Older survivors returned home less often (80%; 73%; 62%, p = 0.05) in favour of transfers to rehabilitation services (10%; 13%; 22%) with higher mortality at 3 months (35%; 32%; 50%, p < 0.0001). CONCLUSION: Older patients hospitalised for severe AKI have a specific profile with more comorbidities, lower baseline renal function, an aetiology of AKI of mainly extra-parenchymal causes and a complex pathway of care with an overall poor prognosis.

Biopsy-proven kidney involvement in hypocomplementemic urticarial vasculitis.

BACKGROUND: Hypocomplementemic urticarial vasculitis (HUV) is a rare systemic vasculitis. We aimed to describe the kidney involvement of HUV in a multicenter national cohort with an extended follow-up. METHODS: All patients with HUV (international Schwartz criteria) with a biopsy-proven kidney involvement, identified through a survey of the French Vasculitis Study Group (FVSG), were included. A systematic literature review on kidney involvement of HUV was performed. RESULTS: Twelve patients were included, among whom 8 had positive anti-C1q antibodies. All presented with proteinuria, from mild to nephrotic, and 8 displayed acute kidney injury (AKI), requiring temporary haemodialysis in 2. Kidney biopsy showed membrano-proliferative glomerulonephritis (MPGN) in 8 patients, pauci-immune crescentic GN or necrotizing vasculitis in 3 patients (with a mild to severe interstitial inflammation), and an isolated interstitial nephritis in 1 patient. C1q deposits were observed in the glomeruli (n = 6), tubules (n = 4) or renal arterioles (n = 3) of 8 patients. All patients received corticosteroids, and 9 were also treated with immunosuppressants or apheresis. After a mean follow-up of 8.9 years, 6 patients had a preserved renal function, but 2 patients had developed stage 3-4 chronic kidney disease (CKD) and 4 patients had reached end-stage kidney disease (ESKD), among whom 1 had received a kidney transplant. CONCLUSION: Renal involvement of HUV can be responsible for severe AKI, CKD and ESRD. It is not always associated with circulating anti-C1q antibodies. Kidney biopsy shows mostly MPGN or crescentic GN, with frequent C1q deposits in the glomeruli, tubules or arterioles.

Characteristics of outpatients referred for a first consultation with a nephrologist: impact of different guidelines.

INTRODUCTION: Chronic kidney disease (CKD) affects > 10% of the population but not all CKD patients require referral to a nephrologist. Various recommendations for referral to nephrologists are proposed worldwide. We examined the profile of French patients consulting a nephrologist for the first time and compared these characteristics with the recommendations of the International Kidney Disease: Improving Global Outcomes (KDIGO), the French "Haute Autorité de Santé" (HAS), and the Canadian Kidney Failure Risk Equation (KFRE). METHODS: University Hospital electronic medical records were used to study patients referred for consultation with a nephrologist for the first time from 2016 to 2018. Patient characteristics (age, sex, diabetic status, estimated glomerular filtration rate (eGFR) and urine protein-to-creatinine ratio (PCR), etiology reported by the nephrologist) and 1-year patient follow-up were analyzed and compared with the KDIGO, HAS and Canadian-KFRE recommendations for referral to a nephrologist. The stages were defined according to the KDIGO classification, based upon kidney function and proteinuria.  RESULTS: The 1,547 included patients had a median age of 71 [61-79] years with 56% males and 37% with diabetes. The main nephropathies were vascular (40%) and glomerular (20%). The KDIGO classification revealed 30%, 47%, 19%, 4% stages G1-2 to G5, and 50%, 22%, 28% stages A1-A3, respectively. According to KDIGO, HAS and KFRE scores, nephrologist referral was indicated for 42%, 57% and 80% of patients respectively, with poor agreement between recommendations. Furthermore, we observed 890 (57%) patients with an eGFR> 30 ml/min and  a urine protein to creatinine ratio 0.5 g/g, mostly aged over 65 years (67%); 40% were diabetic, and 57% had a eGFR > 45 ml/min/1.73m2, 56% were diagnosed as vascular nephropathy and 11% with unknown nephropathy. CONCLUSION: These results underline the importance of better identifying patients for referral to a nephrologist and informing general practitioners. Other referral criteria (age and etiology of the nephropathy) are debatable.

Low performance of prognostic tools for predicting death before dialysis in older patients with advanced CKD.

INTRODUCTION: Chronic kidney disease (CKD) is a disease which is spreading worldwide, especially among older patients. Several prognostic scores have been developed to predict death in older CKD patients, but they have not been validated. We aimed to evaluate the existing risk scores for predicting death before dialysis start, identified via an in-depth review, in a cohort of elderly patients with advanced CKD. METHODS: We performed a review to identify scores predicting death, developed in and applicable to CKD patients. Each score was evaluated with an absolute risk calculation from the patients' baseline characteristics. We used a French prospective multicentre cohort of elderly patients (> 75 years) with advanced CKD [estimated glomerular filtration rate (eGFR) < 20 mL/min/1.73 m2], recruited from nephrological centres, with a 5-year follow-up. The outcome considered was death before initiating dialysis. Discrimination [area under curve (AUC)], calibration and Brier score were calculated for each score at its time frame. RESULTS: Our review found 6 equations predicting death before dialysis in CKD patients. Four of these (GOLDFARB, BANSAL, GRAMS 2 and 4 years) were evaluated. The validation cohort (Parcours de Soins des Personnes Âgées Parcours de Soins des Personnes Âgées, PSPA) included 573 patients, with a median age of 82 years and a median eGFR of 13 mL/min/1.73 m2. At the end of follow-up, 287 (50%) patients had started dialysis and 238 (41%) patients had died before dialysis. The four equations evaluated showed average discrimination (AUC 0.61-0.70) and, concerning calibration, a global overestimation of the risk of death. DISCUSSION: The available scores predicting death before dialysis showed low performance among older patients with advanced CKD in a French multicentre cohort, indicating the need to upgrade them or develop new scores for this population.

Effect of emergency start and central venous catheter on outcomes in incident hemodialysis patients: a prospective observational cohort.

BACKGROUND: Unfavorable conditions at hemodialysis inception reduce the survival rate. However, the relative contribution to outcomes of predialysis follow-up, symptoms, emergency start or central venous catheter (CVC) is unknown. METHODS: We analyzed the determinants of survival according to dialysis initiation conditions in the nationwide REIN registry, using two methods based either on clinical classification or data mining. We divided patients into four groups according to dialysis initiation (emergency vs planned, symptoms or not, previous follow-up). "Followed planned starters" began dialysis as outpatients and with an arteriovenous fistula (AVF). "Followed symptomatic non-urgent starters" were patients who started earlier because of any non-urgent symptomatic event. "Followed urgent starters" had seen a nephrologist before inception but started dialysis in an emergency condition. "Unknown urgent starters" were patients without any follow-up and who had a CVC at inception. RESULTS: "Followed urgent" starters had the lowest 2-year survival rate (66.8%) compared to "followed planned" (77.3%), "followed symptomatic non urgent" (79.2%), and "unknown urgent" (71.7%). Compared to other groups, the risk of mortality was lower in followed symptomatic non urgent (HR 0.86 95% CI 0.75-0.99) and higher in followed urgent starters (HR 1.05 (95% CI 0.94-1.18). In data mining Classification And Regression Tree regrouping in five categories, the lowest 2-year survival (52.3%) was in over 70-year-old starters with a CVC. The survival was 93.2% in under 57-year-old patients without active cancer, 82.5% in 57-70-year-old individuals without cancer, 72.4% in over 70-year-old patients without CVC and 61.4% in under 70-year-old subjects with cancer. The hazard ratio of data mining categories varied between 2.12 (95% CI 1.73-2.60) in 57-70-year-old subjects without cancer and 4.42 (95% CI 3.64-5.37) in over 70-year-old patients with CVC. Therefore, regrouping incident patients into five data mining categories, identified by age, cancer, and CVC use, could discriminate the 2-year survival in patients starting hemodialysis. CONCLUSIONS: Although each classification captured different prognosis information, both analyses showed that starting hemodialysis on a CVC has more dramatic outcomes than emergency start per se.

Diagnostic Performance of Cervical Ultrasound, 99mTc-Sestamibi Scintigraphy, and Contrast-Enhanced 18F-Fluorocholine PET in Primary Hyperparathyroidism.

Preoperative localization of pathologic parathyroids is crucial for minimally invasive treatment of primary hyperparathyroidism (PHPT). This study compared contrast-enhanced 18F-fluorocholine PET/CT, cervical ultrasonography (CU), and conventional scintigraphic imaging modalities (MIBI scintigraphy, consisting of 99mTc-sestamibi/123I-sodium iodide SPECT/CT, 99mTc-sestamibi/123I-sodium iodide planar subtraction imaging, and 99mTc-sestamibi planar dual-phase imaging), combined and individually, for preoperative localization of hyperfunctional parathyroids in PHPT. The gold standard was histologic examination. Methods: Data from consecutive patients with clinically suspected PHPT were retrospectively collected. All 3 imaging modalities were systematically performed. The ability of 18F-fluorocholine PET/CT, CU, and MIBI scintigraphy to identify a hyperfunctional parathyroid and specify the side or identify an ectopic location was noted. Patients underwent surgical exploration if at least 1 examination was positive. The findings of CU + MIBI scintigraphy combined were considered positive if CU and MIBI scintigraphy separately showed a hyperfunctional parathyroid gland on the same side or in the same ectopic location; any findings other than these were considered negative. The composite judgment criterion for pathologic parathyroid was a combination of histologic analysis and normalization of parathyroid hormone and calcium levels. Results: In total, 149 pathologic parathyroids were found in 143 of the 144 included patients. 18F-fluorocholine PET/CT diagnosed 148 of 149 pathologic parathyroids. Only 4 false-positives and 1 false-negative were found. The 18F-fluorocholine PET/CT sensitivity of 99.3% was superior to that of CU, at 75.2% (P < 0.0001); MIBI scintigraphy, at 65.1% (P < 0.0001); and CU + MIBI scintigraphy, at 89.9%, (P = 0.0009). Five of the 5 ectopic locations were diagnosed by 18F-fluorocholine PET/CT, 2 of the 5 by MIBI scintigraphy, and none by CU. Accuracy was better for 18F-fluorocholine PET/CT, at 98%, than for CU, at 84% (P < 0.0001); MIBI scintigraphy, at 81% (P < 0.0001); or CU + MIBI scintigraphy, at 91% (P < 0.0001). Among the 72 (50%) patients who had a negative CU + MIBI scintigraphy result, 18F-fluorocholine PET/CT correctly identified hyperfunctional thyroids in 70 (97.2%). Average uptake in the 18F-fluorocholine PET/CT hyperfunctional parathyroid was higher than that in the adjacent thyroid (SUVmax adjusted for lean body mass, 6.45 vs. 2.15) (P < 0.0001). Conclusion: The accuracy of 18F-fluorocholine PET/CT is higher than that of CU and MIBI scintigraphy for localization of hyperfunctional parathyroids, justifying the systematic use of 18F-fluorocholine PET/CT as the first-line method for PHPT diagnosis.

Apheresis in Adult With Refractory Idiopathic Nephrotic Syndrome on Native Kidneys.

BACKGROUND: Apheresis is the gold standard for idiopathic nephrotic syndrome (INS) relapse after transplantation, but it remains unknown whether such treatment is useful for adults with refractory INS on native kidneys. METHODS: This retrospective study included patients older than 16 years with biopsy-proven refractory (persistent nephrotic syndrome on corticosteroids plus at least 1 immunosuppressive drug) INS treated by apheresis and followed for at least 3 months. RESULTS: Between September 1997 and January 2020, 21 patients (focal segmental glomerulosclerosis: 12, minimal change nephrotic syndrome: 9, men: 67%, median age: 34 years) were identified. At last follow-up (12 months), 7 of 21 patients were in complete or partial remission. Remission was associated with older age (51 vs. 30 years, P = 0.05), lower proteinuria (3.9 vs. 7.3 g/d, P = 0.03), and lower estimated glomerular filtration rate (eGFR) (28.0 vs. 48.5 ml/min per 1.73 m2, P = 0.05) at apheresis. The need for dialysis before apheresis (odds ratio [OR] 22.0 [1.00-524], P = 0.026), age ≥50 years (OR: 22.6 [1.00-524], P = 0.006), a marked (>4.5 g/d) decrease in proteinuria (OR: 9.17 [1.15-73.2], P = 0.041), and a short (<12 months) time between diagnosis and apheresis (OR: 10.8 [1-117], P = 0.043) were significantly associated with remission. Three of 7 patients in remission who were initially on dialysis became dialysis-free; by contrast, none of the 14 patients without remission was initially on dialysis, but 5 of 14 had become dialysis-dependent (P = 0.01). CONCLUSION: Apheresis may result in remission in adult patients with refractory INS, particularly in those at risk of renal failure, with limited sensitivity to medical treatments, if apheresis is initiated within a year of diagnosis.

Two cases of secondary AA amyloidosis involving the skin and chronic kidney infection with a nephrotic syndrome in a high-income country.

We present two French cases of amyloid-associated (AA) amyloidosis secondary to chronic infections. Patient 1, a 51-year-old heroin addict, was hospitalised for chest pain and anasarca. During hospitalisation, a nephrotic syndrome with an inflammatory condition was discovered along with a chronic skin ulcer on his arm. Salivary gland and kidney biopsies confirmed the diagnosis of AA amyloidosis. Renal function quickly declined and haemodialysis was initiated 6 months later. Patient 2, a 55-year-old woman, was hospitalised for obstructive pyelonephritis secondary to coraliform lithiasis. Renal insufficiency with an impure nephrotic syndrome was found. After nephrectomy due to chronic pyelonephritis and an atrophic cortex on the abdominal scan, the histology revealed AA amyloidosis. Despite treatment with ACE inhibitors and control of inflammation, the nephrotic syndrome persisted with rapid decline of the kidney function.

Low performance of prognostic tools for predicting dialysis in elderly people with advanced CKD.

INTRODUCTION: Clinical decision-making about care plans can be difficult for very elderly people with advanced chronic kidney disease (CKD). Current guidelines propose the use of prognostic tools predicting end stage renal disease (ESRD) to assist in a patient-centered shared decision-making approach. Our objective was to evaluate the existing risk model scores predicting ESRD, from data collected for a French prospective multicenter cohort of mainly octogenarians with advanced CKD. METHODS: We performed a rapid review to identify the risk model scores predicting ESRD developed from CKD patient cohorts and evaluated them with data from a prospective multicenter French cohort of elderly (> 75 years) patients with advanced CKD (estimated glomerular filtration rate [eGFR] < 20 mL/min/1.75m2), followed up for 5 years. We evaluated these scores (in absolute risk) for discrimination, calibration and the Brier score. For scores using the same time frame, we made a joint calibration curve and compared areas under the curve (AUCs). RESULTS: The PSPA cohort included 573 patients; their mean age was 83 years and their median eGFR was 13 mL/min/1.73 m2. At the end of follow-up, 414 had died and 287 had started renal replacement therapy (RRT). Our rapid review found 12 scores that predicted renal replacement therapy. Five were evaluated: the TANGRI 4-variable, DRAWZ, MARKS, GRAMS, and LANDRAY scores. No score performed well in the PSPA cohort: AUCs ranged from 0.57 to 0.65, and Briers scores from 0.18 to 0.25. CONCLUSIONS: The low predictiveness for ESRD of the scores tested in a cohort of octogenarian patients with advanced CKD underlines the need to develop new tools for this population.

Humanized anti CD-20 as an alternative in chronic management of relapsing thrombotic thrombocytopenic microangiopathy resistant to rituximab due to anti chimeric antibody.

Acquired Immune thrombotic thrombocytopenic purpura (iTTP) is considered among clinical situations that needs not only urgent treatment in acute setting but also long term management to prevent relapses. Important progresses have been made in management of these patients that are definitely associated with reduced mortality and relapse rate. However, there are still noticeable percentage of patients that may relapse despite application of modern treatment strategies including preemptive rituximab infusions. Hereby, we share our experience concerning a frequently relapsing iTTP due to development of anti-rituximab antibody. In our case administration of obinutuzumab, a humanized type II anti CD-20 antibody was associated with complete peripheral blood B cell depletion and increasing plasma ADAMTS-13 activity.