Introduction: Renal involvement is a severe manifestation of antineutrophil cytoplasmic antibody-associated vasculitis. Patients often progress to end-stage renal disease. The potential for renal recovery after the 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 the eGFR between diagnosis and the follow-up at 3 months (ΔeGFRM0–M3). Methods: This was a retrospective study over the period 2003–2018 of incident patients in the Nord-Pas-de-Calais (France). The primary outcome was the ΔeGFRM0–M3. 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/1.73 m2, p = 0.003). The factors significantly associated with the ΔeGFRM0–M3 in multivariate analysis were the percentage of cellular crescents and neurological involvement. The mean increase in the eGFR was 2.90 ± 0.06 mL/min/1.73 m2 for every 10-point gain in the percentage of cellular crescents. Conclusions: Early renal recovery after the first flare of pauci-immune glomerulonephritis occurred mainly in the first 3 months of treatment. The percentage of cellular crescents was the main independent predictor of early renal recovery.

IntroductionLa population française vieillit. Les études de registre françaises (REIN) montrent également un vieillissement progressif des cohortes de patients incidents dialysés. La prise en charge de patients de plus de 90 ans n’est plus rare et soulève de nombreuses interrogations spécifiques.DescriptionL’ensemble des patients âgés de plus de 90 ans ayant débuté la dialyse (HD et DP) au sein de notre centre entre le 1er janvier 2013 et le 31 décembre 2017 ont été inclus dans l’étude.MéthodesLes données cliniques et paracliniques ont été colligées de façon indépendante et rétrospective. Leur analyse statistique a été effectuée à l’aide d'SPSS.RésultatsDix-sept patients de plus de 90 ans ont débuté la dialyse dans notre centre. Leurs comorbidités sont lourdes (moyenne du score de Charlson de 10 ; 53 % des patients avec au moins 3 comorbidités). Ces patients sont entourés (1/17 est isolé socialement) et peu déprimés (2/17). Tous les patients bénéficiaient d’un suivi régulier en consultation (2 ans environ de suivi moyen). Trente-cinq pour cent avaient bénéficié de la proposition d’un traitement conservateur. Le patient est à l’origine de la demande de dialyse dans 16 cas/17. Pour 35 % des patients, la modalité initiale choisie de dialyse est la DP. Quand l’HD fut pratiquée, elle l’était en centre lourd, majoritairement sur cathéter (82 %). Le programme d’HD était allégé (9h en moyenne). La probabilité de survie à 1 an des patients est estimée à 80 %. Quatre patients ont eu une survie sup à 3 ans. Pour 56 % des patients, le décès survient des suites d’un souhait d’un arrêt de la dialyse. Le temps d’hospitalisation de ces patients est faible (moyenne de 35jours pour 597jours de survie).ConclusionPour des patients sélectionnés, la dialyse au-delà de 90 ans apparaît possible et avec des résultats encourageants.

IntroductionDans notre expérience, plus de 10 % des patients hémodialysés présentent soit une contre indication médicale à l’anticoagulation du circuit soit la nécessité de disposer de méthodes spécifiques et/ou doses optimisées d’anticoagulation afin d’éviter l’apparition de phénomènes thrombotiques du dialyseur. La membrane NV-U est une polysulfone à propriétés hydrophiles proposant une moindre thrombogénicité à la fois en hémodialyse et en hémodiafiltration.DescriptionÉtude prospective monocentrique incluant 10 patients et 33 séances de dialyse réalisées avec la membrane NV-U.MéthodesInclusion de patients âgés de plus de 18 ans hémodialysés depuis plus de 3 mois et présentant : une contre indication à l’anticoagulation du circuit (antécédents hémorragiques n=4), une calciphylaxie n=1, une thrombopénie immunoallergique à l’héparine n=1, des coagulations itératives de circuit traitées par HDF prédilutionnelle et héparine n=4.RésultatsDix patients (9 H – 1 F), âge moyen 72,5a (64–84a), durée moyenne de dialyse 7,2a (2–17a), diabète 6/10, HTA 7/10, obésité 5/10, ratio FAV/KT 5/5.Avant NV-U : 23 séances en HD (22 EVODIAL® – 1 XEVONTA®), 10 séances en HDF prédilutionnelle (3 REXSYS®, 4 XEVONTA®, 3 FX®) – Dose moyenne enoxaparine 50mg (40–100) en HDF.Après NV-U : 2 coagulations massives de circuit survenant à la 3e heure – la 1re en HDF prédilution la 1re séance, aucune récidive en ajoutant de l’enoxaparine 40 et 20mg à T0 et T2 les 2 séances suivantes – la 2e en HD sans anticoagulant, pas de séance de contrôle. Trois séances initiées avec de l’enoxaparine, 1 avec de l’orgaran suivie d’au moins 2 séances sans anticoagulation régionale et sans évènement thrombotique perdialytique.ConclusionAvec seulement 2 coagulations de circuit sur 33 séances, la membrane NV-U semble une option plus qu’intéressante chez des patients chez qui l’anticoagulation est contre indiquée ou complexe, y compris en HDF, à confirmer sur une plus grande cohorte.

Abstract Background and Aims Intravenous etelcalcetide was approved in Europe in 2016 for treatment of secondary hyperparathyroidism (SHPT) in adult patients on hemodialysis (HD). Data on real-world use of etelcalcetide are needed to inform clinicians on routine clinical practice with this newly approved therapy. Method A multi-country observational medical chart review study was performed to describe clinical management of patients treated with etelcalcetide. Sites with at least 6 months of use of etelcalcetide were eligible for study participation. Chronic HD patients who had at least one record of etelcalcetide prescription were recruited. Abstracted data included demographics, clinical history, laboratory parameters and etelcalcetide use over time. Interim data are being reported. Results Medical charts for 238 HD patients who started etelcalcetide between December 24, 2016 and June 7, 2019 were reviewed. Data were obtained from 47 sites from 9 countries (Austria, Denmark, France, Germany, Greece, Netherlands, Russia, Slovenia, and Spain). Forty eight percent of patients (113/238) switched from cinacalcet to etelcalcetide (≤90 days from last cinacalcet prescription), whereas the remaining 125 patients were calcimimetic naive. Median (interquartile range, IQR) age was 62.5 (52-74) and dialysis vintage was 4.7 years (2.4-8.7). Twenty four percent of patients were diabetic and 17% of patients had a history of at least one cardiovascular event (heart failure, myocardial infarction, peripheral vascular disease, or stroke). Parathyroidectomy had been performed in 6% (13/238) of patients and 11% (26/238) had received a kidney transplant. Two-thirds of patients (63%) had a starting etelcalcetide dose of 5 mg and the median weekly dose was 7.5 mg (range: 2.5-15 mg). Table 1 summarizes the median and IQR for parathyroid hormone (PTH), calcium (Ca) and phosphate (P) levels at baseline, 3, 6 and 12 months following etelcalcetide initiation. At baseline, 85% (201/237) had normal Ca (≥2.1 mmol/L). Among patients who had a normal Ca at baseline, the cumulative incidence of hypocalcemia (<2.1 mmol) at 3, 6, 9 and 12 months was, 31%, 45%, 57% and 63%, respectively. Median time to first hypocalcemia event (Kaplan-Meier estimation) was 6.9 months (95% CI: 5.4, 8.9). Etelcalcetide persistence at 3, 6, 9 and 12 months was 96%, 94%, 91% and 87%, respectively. Of the 40 patients who discontinued etelcalcetide by 12 months, 10 patients discontinued for side effects (hypocalcemia=6, nausea=3, and vomiting=1), 5 for parathyroidectomy, 9 for low PTH, 1 for high PTH, and 15 for other reasons. More than half of the patients achieved a >30% reduction in PTH from baseline at 6-months (55.9%) and 73.5% at 12 months; and it was 63.2% and 80% for patients who were calcimimetic naive and 47.8% and 66.4% for patients who switched from cinacalcet to etelcalcetide, respectively. Conclusion To date, this is the largest study on real-world etelcalcetide use in Europe. Etelcalcetide switchers had higher PTH levels than calcimimetic naive patients at initiation. Persistence of etelcalcetide remained high at 12 months. As expected, we observed a substantial reduction in PTH, Ca, and P, and this was greater among patients who were calcimimetic naive than switchers (-43% vs. -32% at 12 months) for PTH. No new safety signals were observed.

RATIONALE & OBJECTIVE Fibrinogen A α-chain amyloidosis (AFib amyloidosis) is a form of amyloidosis resulting from mutations in the fibrinogen A α-chain gene (FGA), causing progressive kidney disease leading to kidney failure. Treatment may include kidney transplantation (KT) or liver-kidney transplantation (LKT), but it is not clear what factors should guide this decision. The aim of this study was to characterize the natural history and long-term outcomes of this disease, with and without organ transplantation, among patients with AFib amyloidosis and various FGA variants. STUDY DESIGN Case series. SETTING & PARTICIPANTS 32 patients with AFib amyloidosis diagnosed by genetic testing in France between 1983 and 2014, with a median follow-up of 93 (range, 4-192) months, were included. RESULTS Median age at diagnosis was 51.5 (range, 12-77) years. Clinical presentation consisted of proteinuria (93%), hypertension (83%), and kidney failure (68%). Manifestations of kidney disease appeared on average at age 57 (range, 36-77) years in patients with the E526V variant, at age 45 (range, 12-59) years in those with the R554L variant (P<0.001), and at age 24.5 (range, 12-31) years in those with frameshift variants (P<0.001). KT was performed in 15 patients and LKT was performed in 4. In KT patients with the E526V variant, recurrence of AFib amyloidosis in the kidney graft was less common than with a non-E526V (R554L or frameshift) variant (22% vs 83%; P=0.03) and led to graft loss less frequently (33% vs 100%). Amyloid recurrence was not observed in patients after LKT. LIMITATIONS Analyses were based on clinically available historical data. Small number of patients with non-E526V and frameshift variants. CONCLUSIONS Our study suggests phenotypic variability in the natural history of AFib amyloidosis, depending on the FGA mutation type. KT appears to be a viable option for patients with the most common E526V variant, whereas LKT may be a preferred option for patients with frameshift variants.

Introduction: We describe the characteristics of patients with moderate/advanced chronic kidney disease (CKD) according to receipt of lipid-lowering therapy (LLT), and whether they achieved low-density lipoprotein cholesterol (LDL-C) targets for high-and very high-risk patients. Methods: CKD-REIN (NCT03381950), a prospective cohort study conducted in 40 nephrology clinics in France, enrolled 3033 patients with moderate (stage G3) or advanced (stage G4/G5) CKD (2013-2016) who had not been on chronic dialysis or undergone kidney transplantation. Data were collected from patients' interviews and medical records. Patients were followed up at 1 year. Results: Among 2542 patients (mean [SD] age 67 [13] years, 34% women) with LDL-C measurements at baseline (mean [SD] LDL-C 2.7 [1.1] mmol/l; cholesterol 4.8 [1.3] mmol/l), 63% were on LLT; 24% were at high (CKD stage G3, no cardiovascular disease [CVD] or diabetes) and 74% at very high (CKD stage G3 with diabetes or CVD, or CKD stage G4/5) cardiovascular risk. Among high-risk patients, 45% of those on statin and/or ezetimibe achieved the LDL-C treatment target (<2.6 mmol/l). Among very high-risk patients, the percentage at goal (<1.8 mmol/l) was 38% for CKD stage G3 and 29% for stage G4/5. There was a trend toward higher achievement of LDL-C targets with increasing LLT intensity (adjusted odds ratios for moderate vs. low intensity 1.20; 95% confidence interval 0.92-1.56; high vs. low intensity 1.46; 1.02-2.09; P-trend = 0.036). Conclusion: Many patients with CKD stage G3-G5 who are eligible for LLT are not treated, and those on LLT rarely achieve LDL-C targets.

Background: Pauci-immune glomerulonephritis (PIGN) is a major prognostic factor in antineutrophil cytoplasmic antibodies-associated vasculitis (AAV). Renal remission is usually defined as improvement or stabilization of serum creatinine and proteinuria levels but the significance of hematuria is unclear. We evaluated the prognostic value of microscopic hematuria in patients in remission from a first flare of PIGN. Methods: A multicenter retrospective study was conducted of all patients with histologically proven PIGN in northern France who presented a first renal flare of AAV between 2003 and 2013. All patients received conventional induction treatment and were considered in remission. Two groups were defined by the presence (H+) or absence (H–) of hematuria (dipstick 1+ and/or cytology ≥10,000 erythrocytes/mL). The primary outcome measure was the occurrence of renal relapse (RR) and/or end-stage renal disease (ESRD). Results: Eighty-six patients were included: 41 (48%) had hematuria at remission. The median follow-up time was 44 ± 34 months. There was no significant difference between the groups in terms of the primary endpoint or the number of RR. However, the survival rate without RR was significantly lower in the H+ group (p = 0.002). In multivariate analysis, risk factors for RR were hematuria at remission for relapses within 44 months (hazard ratio [HR] 4.15; 95% CI 1.15–15.01; p = 0.03) and the duration of maintenance immunosuppressive therapy (HR 0.96 per additional month; 95% CI 0.94–0.99; p = 0.002). Conclusion: Hematuria at remission after a first PIGN flare was not associated with ESRD but with the occurrence of RR within 44 months of remission.

Light chain cast nephropathy is the most common form of kidney disease in patients with multiple myeloma. Light chain casts may occasionally show amyloid staining properties, that is, green birefringence after Congo red staining. The frequency and clinical significance of this intratubular amyloid are poorly understood. Here, we retrospectively assessed the clinicopathological features of 60 patients with histologically proven light chain cast nephropathy with a specific emphasis on intratubular amyloid, especially, its association with extrarenal systemic light chain amyloidosis. We found intratubular amyloid in 17 cases (17/60, 28%) and it was more frequent in patients with λ light chain gammopathy (13/17 in the 'intratubular amyloid' group vs 19/43 in the 'no intratubular amyloid' group, P=0.02). Pathological examination of extrarenal specimens showed that intratubular amyloid was significantly associated with the occurrence of systemic light chain amyloidosis (5/13 in the 'intratubular amyloid' group vs 0/30 in the 'no intratubular amyloid' group, P=0.001). Our results indicate that first, intratubular amyloid is not a rare finding in kidney biopsies of patients with light chain cast nephropathy, and, second, it reflects an amyloidogenic capacity of light chains that can manifest as systemic light chain amyloidosis. Thus, intratubular amyloid should be systematically screened for in kidney biopsies from patients with light chain cast nephropathy and, if detected, should prompt a work-up for associated systemic light chain amyloidosis.

L’atteinte rénale est un facteur pronostique majeur des vascularites associées aux ANCA (VAA). Le traitement des VAA vise la rémission rapide. Sur le plan rénal, la rémission est définie par une diminution ou une stabilité de la créatininémie et de la protéinurie. L’hématurie est d’interprétation difficile. Le but de ce travail est d’évaluer la valeur pronostique de l’hématurie lors la rémission au décours d’un premier épisode de GNEC. Étude rétrospective de patients en 1re poussée de GNEC histologiquement prouvée après obtention de la rémission de la VAA (BVAS = 0) après traitement d’induction conventionnel. L’hématurie est définie par : bandelette urinaire > 1+ et/ou la cytologie urinaire ≥ 10 000 érythrocytes/mL (H + ). Le critère de jugement principal (CDJ) est la rechute rénale (RR) et l’insuffisance rénale chronique terminale (IRCT). La RR est définie par une élévation de la créatininémie et/ou l’apparition d’une hématurie et/ou la confirmation histologique menant à une modification du traitement. L’IRCT est définie par un GFRe < 15 mL/min/1,73m2. Les dossiers de 86 patients ont été analysés : 41 femmes et 45 hommes, âge moyen de 60,5 ± 12,3 ans ; MPO-ANCA : n = 42, PR3-ANCA : n = 39 ; créatinine : 2,9 mg/dL au diagnostic, 1,6 mg/dL lors de la rémission. Il n’y a pas de différence clinique, biologique ou histologique significative entre les patients H+ et H− au diagnostic ou lors de la rémission. Le CDJ est atteint chez 34 patients (26 RR). En analyse de survie, il n’a pas de différence pour le CDJ. En revanche, la survie sans RR est moins bonne dans le groupe H+ (p = 0,002). La RR survient plus précocement dans le groupe H+ que dans le groupe H− (30,3 ± 14,1 vs. 55,7 ± 26,8 mois, p = 0,017). En analyse multivariée, 3 facteurs sont associés à la RR : l’hématurie à la rémission pour les RR dans les 44 premiers mois (p = 0,03), la durée d’immunosuppression (p < 0,001) et le type d’ANCA (PR3+ vs. PR3−) (p = 0,07). Aucune différence n’a été retrouvée entre les 2 groupes concernant le risque d’IRCT. En accord avec les études précédentes, nous n’avons pas montré d’influence de l’hématurie sur le risque d’IRCT (Karras A. et al., 2015), mais un rôle pronostique sur le risque de rechute, en particulier dans les premiers mois suivant la rémission (Rhee R.L. et al., 2016). Cette étude rétrospective souligne l’association entre l’hématurie à la rémission et le risque de RR précoce au décours d’une 1re poussée de GNEC.

Thrombotic microangiopathy (TMA) is a poorly recognized cause of collapsing glomerulopathy. The frequency and significance of collapsing glomerulopathy associated with renal TMA have not been specifically studied in native kidney biopsy specimens. Here we retrospectively documented clinicopathologic features of 53 patients with histologically proven TMA in the native kidney, with special emphasis on changes due to focal segmental glomerulosclerosis (FSGS). Histological TMA was related to hypertensive nephropathy in 21 patients, genetic complement abnormalities in 9, drugs in 7, and to other causes in 16 patients. Almost half (26 patients) presented with arteriolar, 6 with glomerular, and 21 with mixed TMA. Using the Columbia classification system for the 53 patients with histological TMA, 33 had concurrent FSGS lesions with collapsing glomerulopathy the dominant variant in 19 patients (58% of the FSGS cases), not otherwise specified in 9 patients, cellular in 3, and perihilar or tip lesions in 1 patient each. The presence of FSGS was associated with a poor renal prognosis, with no prognostic difference between collapsing glomerulopathy and other FSGS variants. Thus, collapsing glomerulopathy is frequently found in native kidney biopsies with TMA, suggesting that endothelial injury may play an important role in the pathophysiology of FSGS.

La tubulopathie myélomateuse (TM) est la complication rénale la plus fréquente du myélome. La coloration par le Rouge Congo de certains des cylindres myélomateux ou « amylose intratubulaire » (AMIT) est une constatation rare dont la signification est inconnue. En particulier, le lien entre l’AMIT et l’amylose AL systémique avec dépôts parenchymateux rénaux ou extrarénaux n’a jamais été étudié. Le critère d’inclusion était un diagnostic de TM sur PBR entre 2002 et 2012. Une relecture histologique des PBR a été effectuée avec coloration du Rouge Congo pour recherche d’AMIT et/ou de dépôts d’amylose au sein du parenchyme rénal. La recherche d’amylose extra-rénale a été réalisée rétrospectivement par coloration du Rouge Congo sur tout prélèvement histologique (biopsie, pièce opératoire) disponible chez les patients inclus. Les données cliniques ont été recueillies en parallèle. Soixante-huit patients avec TM diagnostiquée sur PBR entre 2002 et 2012 ont été inclus. La relecture histologique a mis en évidence une AMIT chez 18/68 (26,5 %) patients. Dans un cas, l’AMIT était associée à la présence de dépôt d’amylose au sein du parenchyme rénal ; dans 3 autres cas, une amylose rénale était mise en évidence sans AMIT. Une amylose extra-rénale a été mise en évidence chez 5/18 patients avec AMIT et chez 1/50 patients sans AMIT. Au total, des dépôts d’amylose parenchymateux rénaux ou extrarénaux étaient détectés chez 6/18 patients avec AMIT et chez 4/50 patients sans AMIT (OR : 5,56 ; p = 0,017). La recherche d’une amylose AL est importante chez les patients atteints de TM. En effet, sa découverte peut modifier le pronostic et le traitement. Notre étude, qui semble indiquer pour la première fois que l’AMIT est associée à la présence d’une amylose AL systémique, souligne l’importance de la recherche systématique de l’AMIT au cours de la TM et, en cas de détection d’une AMIT, de la réalisation d’un bilan approfondi à la recherche d’une amylose AL.

L’activité de transplantation rénale française est l’une des plus élevées dans le monde : en 2011, 2976 greffes ont été réalisées (45,7 par million d’habitants), et le nombre de patients porteurs d’un greffon fonctionnel est estimé à près de 30 000, soit 44,4 % des 67 270 patients traités pour insuffisance rénale terminale. La part des greffes préemptives est faible : 3,3 % des malades incidents. L’analyse des besoins montre l’augmentation régulière des inscriptions sur la liste d’attente, +5 % par an entre 2006 et 2010, mais avec des disparités importantes selon les âges et entre les régions, liées aux différences d’appréciation des indications de greffes. La médiane d’attente d’un greffon après inscription progresse et a atteint 22,3 mois pour les malades inscrits les quatre dernières années. Elle varie considérablement selon la région et l’équipe d’inscription. L’activité de greffe, malgré sa croissance, est insuffisante pour couvrir l’augmentation de la demande et les patients s’accumulent sur la liste d’attente (9000 en janvier 2012). Cette situation de pénurie est la conséquence de l’offre insuffisante de greffons. Le taux de prélèvement d’organes sur donneur décédé est élevé : 1572 prélèvements en 2011 (24,1 par million d’habitants), mais avec une part chez les sujets âgés qui ne cesse de s’accroître (26 % de donneurs de plus de 65 ans). L’activité de greffe à partir de prélèvements sur donneurs décédés par arrêt cardiaque réintroduite en 2006 se développe progressivement : 65 greffes en 2011. Le nombre de greffes réalisées à partir de donneurs vivants connaît un essor récent, 302 en 2011, soit 10,1 % des greffes. Face à l’augmentation prévisible du nombre de candidats à la greffe, tous les efforts doivent être déployés pour accroître l’offre de greffons, en promouvant l’activité de greffe à partir de donneur vivant, et en maintenant et développant celle de prélèvement sur donneur décédé.Kidney transplantation activity in France is among the most important worldwide: in 2011, 2976 transplants have been performed (47.5 per million population), and the number of patients living with a functional graft is estimated around 30,000, representing 44.7% of all patients (n = 67,270) treated for end-stage renal failure. However, the rate of preemptive kidney transplants remains very low, only 3.3% of incident patients starting renal replacement therapy. The analysis of demand showed a progressive increase in recent years, as demonstrated by the registration rate on the kidney transplantation waiting list, increasing by 5% yearly between 2006 and 2010, but with huge differences according to age categories and regional registration areas, reflecting discrepant appreciations in indications for kidney transplantation. The median waiting time between registration and transplantation increased progressively in recent years, reaching 22.3 months with considerable variations according to regional areas and transplantation teams. Kidney transplantation activity, while increasing continuously, is far to cover the rising demand, and inexorably patients accumulate on the waiting list (around 9000 patients were registered by January 2012). This situation is the consequence of insufficient organ procurement activity. The deceased organ procurement rate remained high: 1572 harvested donors in 2011 (24.1 per million population), but the proportion of older donors rose in recent years, to reach the rate of 26% of donors older than 65 years in 2011. The procurement activity of donors after cardiac arrest was reintroduced in 2006, but increased slowly: 65 transplants were performed in 2011 using kidney procured in non heart-beating donors. The living donor kidney transplantation activity has markedly increased recently: 302 living donor transplantations were performed in 2011, representing 10.1% of the kidney transplantations. Facing the predictable increase in the number of candidates, all efforts should be put together, by increasing the living donor transplantation activity and by supporting and promoting the deceased donor procurement activity.

Renal dysfunction is increasingly recognized as a potential clinical feature of mitochondrial cytopathies such as mitochondrial encephalomyopathy, lacticacidosis and stroke-like episodes (MELAS) syndrome. Five cases of MELAS syndrome with renal involvement from 4 unrelated families are presented in this case series. Three of the 5 patients had a history of maternally-inherited diabetes and/or deafness. Focal and segmental glomerulosclerosis and arteriolar hyaline thickening were the most striking findings on renal biopsy. In addition to clinical presentation with the typical symptoms of MELAS syndrome, genetic testing in these patients identified the A3243G point mutation in the tRNALeu gene of the mitochondrial DNA (mtDNA). The diagnosis of MELAS syndrome was thus considered to be unequivocal. The incidence of kidney disease in MELAS syndrome may be underestimated although a study is required to investigate this hypothesis. As the A3243G mtDNA mutation leads to a progressive adult-onset form of focal segmental glomerulosclerosis (FSGS), screening for the MELAS A3243G mtDNA mutation should therefore be performed especially in patients with maternally-inherited diabetes or hearing loss presenting with FSGS.

Renal dysfunction is increasingly recognized as a potential clinical feature of mitochondrial cytopathies such as mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome. Five cases of MELAS syndrome with renal involvement from 4 unrelated families are presented in this case series. Three of the 5 patients had a history of maternally-inherited diabetes and/or deafness. Focal and segmental glomerulosclerosis and arteriolar hyaline thickening were the most striking findings on renal biopsy. In addition to clinical presentation with the typical symptoms of MELAS syndrome, genetic testing in these patients identified the A3243G point mutation in the tRNA(Leu) gene of the mitochondrial DNA (mtDNA). The diagnosis of MELAS syndrome was thus considered to be unequivocal. The incidence of kidney disease in MELAS syndrome may be underestimated although a study is required to investigate this hypothesis. As the A3243G mtDNA mutation leads to a progressive adult-onset form of focal segmental glomerulosclerosis (FSGS), screening for the MELAS A3243G mtDNA mutation should therefore be performed especially in patients with maternally-inherited diabetes or hearing loss presenting with FSGS.

Background: The association between sarcoidosis and glomerular diseases has not been extensively investigated in a large series and the potential features of this uncommon association remain to be determined. Methods: We retrospectively identified 26 patients with biopsy-proven glomerular lesions that occurred in a sarcoidosis context. Potential remission of glomerular disease and sarcoidosis under specific treatment (steroid and/or immunosuppressive agents) was recorded for all patients. Demographic, clinical and biological characteristics were assessed at the time of kidney biopsy for each patient. Therapeutic data were analyzed for all patients. Results: Glomerular disease occurred after the diagnosis of sarcoidosis in 11 of 26 cases (42%) (mean delay of 9.7 years). In six patients (23%), the glomerulopathy preceded the sarcoidosis diagnosis (mean delay 8 years). In the last nine patients (35%), both conditions occurred simultaneously. The most frequent glomerular disease occurring in sarcoidosis patients was membranous nephropathy in eleven cases. Other glomerular lesions included IgA nephropathy in six cases, focal segmental glomerulosclerosis in four patients, minimal change nephrotic syndrome for three patients and proliferative lupus nephritis in two patients. Granulomatous interstitial nephritis was associated with glomerular disease in six patients and was exclusively found in patients in whom the both disease occurred simultaneously. In nine patients with simultaneous glomerular and sarcoidosis diseases, we observed a strong dissociation between glomerular disease and sarcoidosis in terms of steroid responsiveness. At the end of the follow-up (mean of 8.4 years), six patients had reached end-stage renal disease and three patients had died. Conclusions: A wide spectrum of glomerular lesions is associated with sarcoidosis. The close temporal relationship observed in some patients suggests common causative molecular mechanisms of glomerular injury but complete remission of both diseases in response to exclusive steroid therapy is infrequent.

Les manifestations vasculaires au cours du lupus érythémateux systémique (LES) sont de mécanismes (atteinte inflammatoire ou vascularite, athérosclérose, etc.) et de présentations variés.Nous rapportons l’observation d’un patient de 34 ans avec un LES comportant une atteinte cutanée, articulaire, neurologique et rénale. Il présentait un choc hémorragique sur rupture d’un micro-anévrisme de l’artère hépatique gauche dont l’évolution était favorable après transfusions, traitement immunosuppresseur et hydroxychloroquine.Atteinte rare au cours du LES, une vascularite digestive peut se manifester par des micro-anévrismes notamment hépatiques à l’instar de la panartérite noueuse. Dans la littérature, 10 cas de vascularite des artères digestives avec micro-anévrismes ont été rapportés. L’enjeu principal réside dans un diagnostic précoce pour une prise en charge adaptée et une amélioration du pronostic des patients.The vascular disorders in systemic lupus erythematosus (SLE) result from various mechanisms and presentations (inflammatory disease or vasculitis, atherosclerosis).We report on a 34-year-old man with cutaneous, articular, neurological and nephrologic SLE. He presented with catastrophic haemorrhage on microaneurysm rupture of the left hepatic artery. After blood transfusions and immunosuppressive treatments, his condition improves.Uncommon complication in SLE patients, digestive vasculitis with microaneurysms may occur as in polyarteritis nodosa. In the literature, we identified 10 additional cases of hepatic microaneurysms in SLE patients. The main issue is an earlier diagnosis in order to give appropriate treatment and improve prognosis.