Hypertension artérielle maligne (étude rétrospective dans un service de néphrologie)

Etude rétrospective comportant 49 patients, 41 hommes et 8 femmes, adressés dans un service de néphrologie dans une banlieue parisienne. L hypertension artérielle maligne est une vascularite nécrosante d évolution aigue ou subaigue. Cinquante sept pourcent des patients étaient d ethnie africaine, 33% caucasien, 8% asiatique et 2% originaire du Maghreb. Soixante treize pourcent des patients avaient des antécédents d hypertension (HTA) mais seulement 47% avaient un traitement à leur prise en charge. La durée d évolution d hypertension avant la phase maligne était très variable: moyenne 8,2 ans+-6,8 ans, médiane 6,1 ans. Le délai entre présentation et prise en charge était très variable : dix huit pourcent étaient pris en charge le jour même de présentation, moyenne de 45 semaines +-187, médiane 22 semaines. Deux sous populations pathologiques émergent: 95,5% des noirs présentent une hypertension essentielle et 62,5% des blancs une hypertension secondaire, différence significative, x2=15,72, p<O,OO1. La présentation clinique était polymorphe, riche en signes fonctionnels et peu spécifiques exprimant l atteinte des différents systèmes fonctionnels. On propose qu une meilleure connaissance de la population à risque et de l expression clinique puisse améliorer le dépistage précoce et la prise en charge de l hypertension maligne.PARIS13-BU Serge Lebovici (930082101) / SudocSudocFranceF

Granulomatoses rénales (40 observations dans le Nord-Est parisien)

Nous rapportons 40 observations de granulomatoses rénales dans le Nord-Est Parisien, recueillies à partir du registre de 3 services d anatomopathologie survenues entre 01/1991 et 02/2004 dans 1 service de Médecine Interne et 3 services de Néphrologie. Le critère d inclusion était la présence dans l interstitium rénal de granulomes épithélioïdes. Il s agissait de 25 hommes (âge médian: 46 ans; extrêmes, 29 à 74 ans) et 15 femmes (64; 26-78) d origine européenne (23 cas), nord-africaine (7), africaine subsaharienne (6), antillaise (2) et asiatique (2). L étude histologique rénale montrait des granulomes épithélioïdes de l interstitium rénal (100%) associés à une glomérulonéphrite proliférative extra-capillaire pauci-immune (5%) et une vascularite granulomateuse (2,5 %). La présentation néphrologique comprenait une insuffisance rénale (100%) (Créatinine médiane: 236,8 124-805 jimolll) associée à une HTA (27,5%), une protéinurie (100%) (0,6gr/l ; 0,08-3), une hématurie microscopique (15%) et une leucocyturie (22,5%). Les principales manifestations extra-rénales étaient un amaigrissement (30%), une toux sèche (25%), des arthralgies (17,5%), une uvéite (12,5%) et une parotidomégalie (5%). Vingt-trois patients (57,5%) ont eu un total de 28 biopsies extra-rénales. Le diagnostic étiologique était une sarcoïdose dans 20 cas, une origine médicamenteuse dans 7 cas, une tuberculose dans 3 cas, une maladie de Wegener dans 2 cas, une lèpre dans 1 cas, une infection à Mycobactérium avium dans 1 cas, une maladie de Crohn dans 1 cas et pas d étiologie dans 5 cas. Le traitement a comporté: des assauts de méthylprednisolone dans 4 cas de sarcoïdose, 2 cas de Wegener et 3 cas d idiopathique; une corticothérapie orale dans 20 cas de sarcoïdose, 5 cas d immunoallergie, 3 cas d idiopathique, 2 cas de Wegener, 1 cas de tuberculose et de maladie de Crohn; du cyclophosphamide dans 2 cas de Wegener; de l a.zathioprine dans 2 cas de Wegener et un cas de Crohn. L évolution rénale fut favorable dans 15 cas de sarcoïdose, 5 cas d origine médicamenteuse immunoallergique, 3 cas de tuberculose, 1 cas de Wegener, de lèpre, d infection à M.avium, de Crohn et 4 cas d idiopathique.A partir des différentes observations, nous avons discuté la stratégie diagnostique et thérapeutiquePARIS13-BU Serge Lebovici (930082101) / SudocSudocFranceF

Can empathy be taught? A cross-sectional survey assessing training to deliver the diagnosis of end stage renal disease

International audienceBackground: Receiving the diagnosis of kidney failure has a major impact on patients. Yet, the way in which this diagnosis should be delivered is not formally taught within our medical curriculum. To fill this gap we set up a training course of kidney failure diagnosis delivery for nephrology trainees since 2016. This study assessed the effectiveness of this educational intervention.Methods: The primary outcome was change in the empathy score immediately after the training session and several months afterward, based on the Jefferson Scale of Physician Empathy (JSPE). Self-reported change in clinical practice was also evaluated. As control groups, we assessed empathy levels in untrained nephrology trainees (n = 26) and senior nephrologists (n = 71). Later on (>6 months) we evaluated participants’ perception of changes in their clinical practice due to the training.Results: Six training sessions permitted to train 46 trainees. Most respondents (76%) considered the training to have a durable effect on their clinical practice. Average empathy scores were not significantly different in pre-trained trainees (average JSPE: 103.7 ± 11.4), untrained trainees (102.8 ± 16.4; P = 0.81) and senior nephrologists (107.2 ± 13.6; P = 0.15). Participants’ empathy score significantly improved after the training session (112.8 ± 13.9; P = 0.003). This improvement was sustained several months afterwards (average JSPE 110.5 ± 10.8; P = 0.04).Conclusion: A single 4-hour training session can have long lasting impact on empathy and clinical practice of participants. Willingness to listen, empathy and kindness are thought to be innate and instinctive skills, but they can be acquired and should be taught

Renal Lesions Associated with IgM-Secreting Monoclonal Proliferations: Revisiting the Disease Spectrum

Background and objectives: Since the first description of pathology of the kidney in Waldenström disease in 1970, there have been few reports on kidney complications of IgM-secreting monoclonal proliferations. Here, we aimed to revisit the spectrum of renal lesions occurring in patients with a serum monoclonal IgM

The Exostosin Immunohistochemical Status Differentiates Lupus Membranous Nephropathy Subsets With Different Outcomes

International audienceExostosin (EXT) 1 and EXT2 proteins are recently discovered major components of the subepithelial deposits in a subset of patients with membranous nephropathy (MN).1,2 Although the detection of EXT1/2 seems to be a distinctive feature of MN related to systemic lupus erythematosus (SLE),1 the exact frequency of EXT-positive, SLE-associated MN (SLE-MN) and its potential clinical significance are not well determined.Here, we retrospectively evaluated 86 consecutive patients with biopsy-proven pure class 5 SLE-MN diagnosed between January 2010 and December 2018 in 2 nephropathology centers (La Pitié Salpêtrière and Tenon hospitals) in Paris, France; mixed classes (3 + 5 and 4 + 5 of the 2003 ISN/Renal Pathology Society classification)3 were excluded

Management of severe renal disease in anti-neutrophil-cytoplasmic-antibody-associated vasculitis: the place of rituximab and plasma exchange?

Abstract Objective The optimal induction therapy for severe glomerulonephritis of ANCA-associated vasculitis (AAV) is debated. We compared the efficacy of glucocorticoid and rituximab (RTX) or CYC induction therapy for severe AAV-related glomerulonephritis and evaluated the potential benefit of plasma exchange (PE) as adjunct therapy to CYC. Methods This retrospective, multicentre study included AAV patients with severe renal active disease (serum creatinine level ≥350 µmol/l and/or estimated glomerular filtration ratio ≤15 ml/min/1.73 m2). Propensity-score analysis was used to adjust for potential confounders. Results Between 2005 and 2017, 153 patients with AAV-related glomerulonephritis were studied (96 [60%] men; mean [s.d.] age 63 [13.1] years): 19 (12%) were treated with RTX and 134 (88%) with CYC. Remission rates did not differ between RTX- and CYC-treated groups. Although more patients with RTX than CYC were dialysis-free at month (M) 12 (79% vs 68%), the difference was not significant after adjustment. Among 134 patients with CYC-treated glomerulonephritis, 76 (57%) also had PE. M3 and M6 remission rates were comparable for weighted CYC groups with or without PE. For weighted groups, the dialysis-free survival rate with CYC was higher with than without PE at M6 (72% vs 64%; odds ratio 2.58) and M12 (74% vs 60%; odds ratio 2.78) reaching statistical significance at M12. Conclusion We could not find any difference between RTX and CYC as induction therapy for patients with severe AAV-related glomerulonephritis. In patients receiving CYC induction regimen, the addition of PE conferred short-term benefits with higher dialysis-free rate at M12

Rituximab in Severe Lupus Nephritis: Early B-Cell Depletion Affects Long-Term Renal Outcome

Background and objectives: Standard treatment for lupus nephritis, including corticosteroids and cyclophosphamide, is efficient but is still associated with refractory or relapsing disease, or severe deleterious effects. Rituximab, a monoclonal chimeric anti-B cell antibody, is increasingly used in patients with lupus nephritis, but reported series were small and had a short follow-up

Imported Malaria, Collapsing Glomerulopathy, and Focal and Segmental Glomerulosclerosis

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Prognostic Factors in Anti-glomerular Basement Membrane Disease: A Multicenter Study of 119 Patients

International audienceWe report the overall and renal outcome in a French nationwide multicenter cohort of 119 patients with anti-glomerular basement membrane (anti-GBM) disease. Sixty-four patients (54%) had an exclusive renal involvement, 7 (6%) an isolated alveolar hemorrhage and 48 (40%) a combined renal and pulmonary involvement. Initial renal replacement therapy (RRT) was required in 78% of patients; 82% received plasmapheresis, 82% cyclophosphamide, and 9% rituximab. ANCA positive (28%) patients were older (70 vs. 47 years, p < 0.0001), less frequently smokers (26 vs. 54%, p = 0.03), and had less pulmonary involvement than ANCA- patients. The 5 years overall survival was 92%. Risk factors of death (n = 11, 9.2%) were age at onset [HR 4.10 per decade (1.89–8.88) p = 0.003], hypertension [HR 19.9 (2.52–157 0.2) p = 0.005], dyslipidemia [HR 11.1 (2.72–45) p = 0.0008], and need for mechanical ventilation [HR 5.20 (1.02–26.4) p = 0.047]. The use of plasmapheresis was associated with better survival [HR 0.29 (0.08–0.98) p = 0.046]. At 3 months, 55 (46%) patients had end-stage renal disease (ESRD) vs. 37 (31%) ESRD-free and 27 (23%) unevaluable with follow-up < 3 months. ESRD patients were older, more frequently female and had a higher serum creatinine level at presentation than those without ESRD. ESRD-free survival was evaluated in patients alive without ESRD at 3 months (n = 37) using a landmark approach. In conclusion, this large French nationwide study identifies prognosis factors of renal and overall survival in anti-GBM patients

Renal involvement in eosinophilic granulomatosis with polyangiitis (EGPA): a multicentric retrospective study of 63 biopsy-proven cases

International audienceAbstract Objective Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small-vessel vasculitis characterized by asthma, hypereosinophilia and ANCA positivity in 40% of patients. Renal involvement is rare and poorly described, leading to this renal biopsy-proven based study in a large EGPA cohort. Methods We conducted a retrospective multicentre study including patients fulfilling the 1990 ACR criteria and/or the 2012 revised Chapel Hill Consensus Conference criteria for EGPA and/or the modified criteria of the MIRRA trial, with biopsy-proven nephropathy. Results Sixty-three patients [27 women, median age 60 years (18–83)] were included. Renal disease was present at vasculitis diagnosis in 54 patients (86%). ANCA were positive in 53 cases (84%) with anti-MPO specificity in 44 (83%). All patients had late-onset asthma. Peripheral neuropathy was present in 29 cases (46%), alveolar haemorrhage in 10 (16%). The most common renal presentation was acute renal failure (75%). Renal biopsy revealed pauci-immune necrotizing GN in 49 cases (78%). Membranous nephropathy (10%) and membranoproliferative GN (3%) were mostly observed in ANCA-negative patients. Pure acute interstitial nephritis was found in six cases (10%); important interstitial inflammation was observed in 28 (44%). All patients received steroids with adjunctive immunosuppression in 54 cases (86%). After a median follow-up of 51 months (1–296), 58 patients (92%) were alive, nine (14%) were on chronic dialysis and two (3%) had undergone kidney transplantation. Conclusion Necrotizing pauci-immune GN is the most common renal presentation in ANCA-positive EGPA. ANCA-negative patients had frequent atypical renal presentation with other glomerulopathies such as membranous nephropathy. An important eosinophilic interstitial infiltration was observed in almost 50% of cases