Introduction: Although anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is rare in children, kidney involvement is both common and potentially severe. Data on the clinico-pathological presentation and progression of kidney involvement in pediatric AAV are limited. Methods: This multicentric, retrospective, observational study aims to characterize kidney involvement in pediatric AAV, through a centralized pathology review of kidney biopsies and comparisons with an adult AAV cohort. Results: Eighty-one pediatric patients (median age 12.7 years, 23% male) were included over 20 years. Compared with adults (median age 66 years, 52% male), children presented with more frequent nephrotic-range proteinuria (42% vs. 18%, P < 0.001), lower hemoglobin levels (8.7 vs. 9.7 g/dl, P < 0.001), and more common cutaneous (24% vs. 9%, P = 0.001) and gastrointestinal involvement (18% vs. 3%, P < 0.001); histologically, more crescentic (P < 0.001) and fewer focal forms (P < 0.001), less interstitial fibrosis and tubular atrophy (IF/TA 0: 41% vs. 19%, P = 0.001) and more interstitial inflammation (ti1-2-3 per Banff classification: 66% vs. 47%, P = 0.025). For induction treatment children had a more frequent use of rituximab (65% vs. 43%, P = 0.001) and plasma exchange (31% vs. 15%, P = 0.004). Kidney outcomes were comparable to adults. The Berden classification, ANCA Renal Risk Score (ARRS), and ANCA Kidney Risk Score (AKRiS) showed good predictive accuracy for pediatric patients. Repeat biopsies demonstrated reduced activity and progression towards fibrosis. Conclusion: Pediatric patients present a more active kidney histology than adults, a more frequent nephrotic-range proteinuria and more severe anemia at presentation. Despite this, outcomes are comparable to adults. Berden, ARRS, and AKRiS can be used for predicting kidney prognosis in the pediatric population.
BACKGROUND AND OBJECTIVE:Multiparametric magnetic resonance imaging (mpMRI) with targeted biopsies improves detection of clinically significant prostate cancer (csPC), commonly defined as Gleason grade group (GG) ≥2. Current practice combines targeted and systematic biopsies, which increases csPC detection but also increases detection of insignificant PC (GG 1), which contributes to overdiagnosis. Perilesional sampling around MRI-visible lesions has been proposed as a strategy to mitigate targeting imprecision while limiting sampling outside the MRI lesion area and GG 1 detection. The primary objective is to determine the diagnostic performance of the experimental targeted + perilesional biopsy scheme for detection of csPC in comparison to the standard targeted + systematic biopsy scheme. CLINICAL TRIAL DESIGN AND TIMEFRAME:TARGET is a prospective, multicentre, open-label, comparative clinical trial. Each patient acts as their own control, as each patient will undergo all three types of biopsy (targeted, perilesional, and systematic). The inclusion period will last for 21 mo, and the participation duration for each patient is 3 mo. ENDPOINTS:The primary endpoint is the sensitivity and specificity of the targeted + perilesional scheme for csPC detection in comparison to the targeted + systematic scheme. Secondary endpoints include differences in the detection rate for insignificant PC (GG 1) and aggressive PC (GG ≥3) between the experimental and standard biopsy schemes. DATA SOURCES AND STATISTICAL ANALYSIS PLAN:The data collected will include patient demographics and laboratory, radiology, and pathology reports. Analyses will be performed with SAS version 9.4 using a locked database. STRENGTHS AND LIMITATIONS:Strengths include the prospective and multicentre design. The main limitation is the open-label design. FUNDING:TARGET is funded by Ramsay Générale de Santé (Paris, France), and supported by the Prostate Cancer Committee of Association Française d'Uologie. ETHICS AND TRIAL REGISTRATION:The trial was assessed by the CPP Ouest VI ethics committee and is registered on ClinicalTrials.gov as NCT07296042. PATIENT SUMMARY:Our multicentre trial is comparing two different approaches for prostate biopsy to determine if sampling the area around lesions seen on an MRI (magnetic resonance imaging) scan can maintain the detection rate for clinically significant prostate cancer while reducing detection of low-risk disease.
Introduction In ANCA-associated vasculitis (AAV), both the disease and its treatments contribute to systemic complications, among which, infections represent a major cause of morbidity and mortality. This study aimed to evaluate the prevalence and prognosis of infectious complications in AAV, to identify associated risk factors, and to describe long-term outcomes. Methods This retrospective monocentric study included patients followed in the Nephrology and Internal Medicine departments at Conception Hospital after a first episode or relapse of AAV between 2004 and 2024. Infection was considered if requiring hospitalization or an anti-infectious therapy. Follow-up was conducted until January 1st, 2025. Results Among 264 patients included, the incidence of infection was 43.9%, with respiratory infections accounting for 49.1% of cases and bacterial infections for 75.3%, mainly with gram negative bacillus. Infection episodes were significantly associated with age >65 years (p =0.02), hypertension (p=0.04), diabetes (p =0.01), a high Five-Factor Score (p =0.005), a high BVAS (p =0.03), anti-PR3 positivity (p =0.03), acute kidney injury (p=0.004), cyclophosphamide (p =0.05), and intravenous corticosteroid for induction (p =0.03). In multivariate analysis, anti-PR3 positivity (aOR = 2.0, p=0.03) was independent risk factors, whereas methotrexate appeared protective (aOR = 0.08, p=0.001). Infections were independently linked to vasculitis relapses (aOR = 2.8, p=0.007) and cardiovascular events (aOR 3.0, p =0.01). Survival analysis demonstrated a significant association between mortality and infection (HR 2.14 95%CI[1.59-2.91], p<0.001). Conclusion Infections are frequent complications in AAV, and exert an independent impact on mortality. Anti-PR3 positivity increases infection risk, whereas methotrexate appears protective.
OBJECTIVES:Few recent epidemiological data are available on the risks of chronic kidney disease (CKD), cardiovascular events and infections, in patients with LN, even though these data are crucial for guiding therapeutic decisions. We conducted a cohort study with the aim of evaluating survival without CKD and adverse events in patients with LN. METHODS:All patients with a first flare of biopsy-proven LN between 2001 and 2022 followed-up in the University Hospital of Marseille, France, were included in MassiLUP. Patient survival, survival without CKD stages 3, 4 and 5, without cardiovascular event, without severe infection (hospitalization or zoster) and without cancer were assessed. RESULTS:One hundred and sixty-eight patients (82.7% female) were included, mean follow-up was 9.6 ± 5.7 years. Most patients had class III or IV (+/-V) LN. Four patients died (three severe infections and one stroke); 41 (24.4%) patients developed CKD, among whom 19 (11.3%) reached ESKD; 34 (20.2%) patients presented a cardiovascular event, 59 (35.1%) a severe infection and 12 (7.1%) a cancer. Survival rates without CKD were 82.9% and 74.6% at 5 and 10 years; without cardiovascular event were 85.7% and 77.3% at 5 and 10 years; without severe infection were 76.3% and 63.8% at 5 and 10 years; without cancer were 96.3% and 92.9% at 5 and 10 years. CONCLUSION:Despite free access to treatment and care in France, the long-term prognosis of patients with LN remains burdened both by the risk of CKD, and the risks of cardiovascular and infectious events.
Luspatercept is a recombinant fusion protein that enhances late-stage erythropoiesis by inhibiting select transforming growth factor β (TGFβ) superfamily ligands. It is approved for transfusion-dependent β-thalassemia and myelodysplastic syndromes with ring sideroblasts. Kidney toxicity has been rarely reported in humans, although glomerular lesions have been described in preclinical studies. We report the case of a 74-year-old woman with myelodysplastic syndrome with ring sideroblasts treated with luspatercept for persistent anemia. She developed acute kidney injury, glomerular-range proteinuria, microscopic hematuria, and leukocyturia. Renal biopsy revealed a diffuse mesangial sclerosing glomerulopathy characterized by prominent mesangial matrix expansion with minimal proliferative changes, no immune complex deposits on immunofluorescence or electron microscopy. Luspatercept was discontinued, leading to a partial improvement in renal function but persistent proteinuria. This case documents a non-immune mesangial sclerosing glomerulopathy temporally associated with luspatercept therapy. Only one other case of biopsy-proven renal injury linked to luspatercept has previously been published, with an immune complex-mediated MPGN. Clinicians should be aware of possible renal involvement during luspatercept treatment and consider systematic monitoring of kidney function and urinalysis in treated patients. Further data are needed to clarify the spectrum and mechanisms of renal adverse events associated with this drug.
ANCA-associated vasculitis is a rare autoimmune disease, and is particularly serious when the kidneys are involved. In addition to immunosuppressants, plasma exchanges are used to treat severe forms of vasculitis. The international guidelines recommend kidney biopsies for their diagnostic and prognostic roles. The Berden, ARRS (ANCA Renal Risk Score), and AKRiS (ANCA Kidney Risk Score) classifications show that certain histological lesions, such as interstitial fibrosis, tubular atrophy, and the percentage of normal glomeruli negatively impact kidney prognosis. The aim of this study was to evaluate the renal response of patients with severe forms of ANCA-associated vasculitis undergoing kidney biopsy and plasma exchanges, to identify the histological determinants favorable to kidney recovery. This was a retrospective multicenter study of 34 patients with ANCA-associated vasculitis with severe kidney involvement who were receiving plasma exchanges. The primary endpoint was death, the need for dialysis, or persistent kidney dysfunction (eGFR < 30 ml/min/1.73 m² for 3 months) at one year. Among the 34 patients (56
BACKGROUND AND HYPOTHESIS:The diagnosis of kidney disease can be challenging during pregnancy. A kidney biopsy (KB) may be proposed, but there is little recent data available to assess the risk/benefit ratio of this procedure during pregnancy. METHODS:In this French nationwide survey, we analysed the indications, complications, histological diagnoses, treatments, and obstetric outcomes of pregnant women who underwent native KB between 2006 and 2025. RESULTS:We gathered the medical records of 76 patients, with a median age of 29.5 [range 18-42] years, including 2 with twin pregnancies. KB was performed at a median gestational age of 13.5 [range 4-26] weeks. The main indications for KB were: nephrotic syndrome without AKI (40.8%) and non-nephrotic proteinuria without AKI (40.8%). KB led to a diagnosis in 72 (94.7%) patients. The main histological diagnoses were lupus nephritis (43%), focal segmental glomerulosclerosis (13%), membranous nephropathy (13%), IgA nephropathy (12%), and idiopathic nephrotic syndrome consistent with minimal change disease (5%). One patient experienced bleeding requiring transfusion. Specific treatment was initiated in 48 (63.2%) patients after KB: corticosteroids (35.5%), hydroxychloroquine (27.6%), azathioprine (18.4%), calcineurin inhibitor (13.2%), rituximab (2.6%) and belimumab (1.3%). There was a high incidence of preeclampsia (16.4%), small for gestational age (21.3%) and intrauterine fetal death (9.0%) in this cohort. Preterm birth and low birth weight occurred in 65.0% and 56.7% of cases, respectively. CONCLUSIONS:When considered necessary to establish a diagnosis during pregnancy, kidney biopsy performed in the first two trimesters appears to be safe, with a high diagnostic yield and a significant impact on therapeutic decision-making and patient management.
RationaleCOVID-19-associated acute-respiratory distress syndrome (C-ARDS) results from a direct viral injury associated with host excessive innate immune response mainly affecting the lungs. However, cytokine profile in the lung compartment of C-ARDS patients has not been widely studied, nor compared to non-COVID related ARDS (NC-ARDS).ObjectivesTo evaluate caspase-1 activation, IL-1 signature, and other inflammatory cytokine pathways associated with tissue damage using post-mortem lung tissues, bronchoalveolar lavage fluids (BALF), and serum across the spectrum of COVID-19 severity.MethodsHistological features were described and activated-caspase-1 labeling was performed in 40 post-mortem biopsies. Inflammatory cytokines were quantified in BALF and serum from 19 steroid-treated-C-ARDSand compared to 19 NC-ARDS. Cytokine concentrations were also measured in serum from 128 COVID-19 patients at different severity stages.Measurements and main resultsTypical “diffuse alveolar damage” in lung biopsies were associated with activated caspase-1 expression and vascular lesions. Soluble Caspase-1p20, IL-1β, IL-1Ra, IL-6 and at lower level IFNγ and CXCL-10, were highly elevated in BALF from steroid-treated-C-ARDS as well as in NC-ARDS. IL-1β appeared concentrated in BALF, whereas circulating IL-6 and IL-1Ra concentrations were comparable to those in BALF and correlated with severity. TNFα, TNFR1 and CXCL8 however, were significantly higher in NC-ARDS compared to C-ARDS, treated by steroid.ConclusionsIn the lungs of C-ARDS, both caspase-1 activation with a predominant IL-1β/IL-6 signature and IFNγ -associated chemokines are elevated despite steroid treatment. These pathways may be specifically targeted in ARDS to improve response to treatment and to limit alveolar and vascular lung damage.
Quiz focusing on the cytological analysis of urine in a case of urinary tract infection caused by E. coli, and its management with ceftriaxone treatment.
Luspatercept is a recombinant fusion protein that enhances late-stage erythropoiesis by inhibiting select transforming growth factor β (TGFβ) superfamily ligands. It is approved for transfusion-dependent β-thalassemia and myelodysplastic syndromes with ring sideroblasts. Kidney toxicity has been rarely reported in humans, although glomerular lesions have been described in preclinical studies.We report the case of a 74-year-old woman with myelodysplastic syndrome with ring sideroblasts treated with luspatercept for persistent anemia. She developed acute kidney injury, glomerular-range proteinuria, microscopic hematuria, and leukocyturia. Renal biopsy revealed a diffuse mesangiocapillary glomerulopathy with marked mesangial matrix expansion and no immune complex deposits on immunofluorescence or electron microscopy. Luspatercept was discontinued, leading to a partial improvement in renal function but persistent proteinuria.This case documents a non-immune mesangiocapillary glomerulopathy temporally associated with luspatercept therapy. Only one other case of biopsy-proven renal injury linked to luspatercept has previously been published, with a different histopathological pattern. Clinicians should be aware of possible renal involvement during luspatercept treatment and consider systematic monitoring of kidney function and urinalysis in treated patients. Further data are needed to clarify the spectrum and mechanisms of renal adverse events associated with this drug.
Introduction:The identification of prognostic factors for renal failure in antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) remains a challenge. The benefit of plasma exchange (PLEX) has been questioned, and the target population remains to be defined. We investigated the outcome of patients requiring renal replacement therapy (RRT) at baseline and factors associated with their prognosis at 1 year. Methods:This retrospective multicenter study evaluated the 1-year composite end point of death or end-stage kidney disease (ESKD) in patients with biopsy-proven renal AAV involvement. Results:Of the 394 patients included, 105 (26.6%) were on dialysis at baseline. Of these, 60 (57.1%) reached the composite end point compared with 29 patients (10.0%) who were not on RRT at baseline (P < 0.001). On multivariate analysis, age and sex were not associated with the composite outcome (P = 0.945 and P = 0.154, respectively); however, myeloperoxidase (MPO)-ANCA was (odds ratio [OR]: 3.60; 95% confidence interval [CI]: 1.79-7.60), as was a high baseline histologic renal risk score (OR: 1.29; 95% CI 1.17-1.44). The most strongly associated factor remained the need for dialysis at baseline (OR: 10.91; 95% CI: 5.52-22.70). Of the 91 patients surviving after requiring dialysis at baseline, 45 were weaned from RRT (49.5%) at 1 year, and PLEX was independently associated with a reduced risk of the composite outcome (OR: 0.23, 95% CI: 0.05-0.80). Conclusion:MPO-ANCA, need for dialysis, and high histological renal risk score at baseline were associated with the 1-year composite end point of death or ESKD. Almost half of the patients on dialysis at baseline were off dialysis at 1 year, with a better prognosis in those who had received PLEX.
Introduction:Kidney involvement is underestimated in patients with hereditary transthyretin amyloidosis (ATTRv), and few data are available about the renal outcomes of patients treated with targeted therapies. Methods:Patients with ATTRv nephropathy (ATTRv-N) from 6 French referral centers were retrospectively included. The evolution of estimated glomerular filtration rate (eGFR) and proteinuria, and the specific treatments of ATTRv were collected. Renal survival was assessed by using a renal composite end point, including an eGFR decline > 50% from baseline and/or dialysis requirement. Results:Twenty-three patients (70% female) with a median age at ATTRv-N diagnosis of 50 (interquartile range [IQR]: 37-63) years were included. Baseline eGFR was 60 (39-83) ml/min per 1.73 m2. Median urine protein-to-creatinine ratio (UPCR) was 100 (IQR: 20-240) mg/mmol. ATTRv-N was documented by kidney biopsy in 20 of 23 patients (87%). Eleven patients were treated with the transthyretin (TTR) stabilizer, tafamidis; 6 patients with a small interfering RNA (siRNA); 4 with exclusive orthotopic liver transplantation (OLT); whereas 2 received no specific treatment. After a median follow-up of 5.8 (IQR: 3.3-18.6) years, all patients with OLT or no treatment had a progressive eGFR decline, requiring dialysis in 3 of 6 patients. Among patients treated with tafamidis, 8 of 11 (73%) had a progressive eGFR decline, requiring dialysis in 1 patient; and proteinuria was either stable or increasing over time in all patients. All patients who received siRNA therapy had stable or improving eGFR. Renal survival was 44%, 44%, and 100% at 60 months for the patients with exclusive OLT or no treatment, TTR stabilizers, and siRNA, respectively (P = 0.12). Four patients with baseline nephrotic syndrome or high-grade proteinuria, including 3 patients resistant to tafamidis, responded dramatically to siRNA therapy, with a fast, complete, and sustained remission of proteinuria within 1 year. Conclusion:Our study highlights the underrecognized risk of chronic kidney disease (CKD) and end-stage kidney disease in ATTRv and suggests that siRNA could be a promising therapeutic option for the stabilization of kidney function.
C3 glomerulonephritis (C3GN) is characterized by glomerular aggression mediated by deregulation of the alternative complement pathway. C3GN can be inherited or consequent to acquired autoantibodies, notably against factor H. We report the case of a patient with systemic active IgG4-related disease who presented for acute kidney injury with glomerular proteinuria and hypocomplementemia related to C3GN associated with IgG4-related interstitial nephritis on kidney biopsy. Factor H was low, and antifactor H IgG autoantibody was detected. Detection of other acquired or genetic complement alternative pathway disorders returned negative. After initial failure of oral corticoids and intravenous rituximab, the patient was successfully treated by intravenous cyclophosphamide followed by maintenance therapy with rituximab. Antifactor H autoantibody isotypes were IgG1 and IgG3, mainly as all antifactor H in positive controls but also IgG4, which is unusual. This suggests a link in this case between the oligoclonal expansion of plasma cells in IgG4-related disease and the production of antifactor H antibodies, especially of IgG4 isotype.
Background: Scleroderma renal crisis is a severe complication of systemic sclerosis that is associated with higher morbidity and mortality. However, limited data are currently available regarding the factors affecting renal outcome during scleroderma renal crisis. The objective of this study is to describe renal histopathology in scleroderma renal crisis and to evaluate its association with kidney failure. Methods: We performed a French multicenter retrospective study that included 65 patients who underwent a kidney biopsy in the context of scleroderma renal crisis, between 2006 and 2020. Non-supervised hierarchical cluster analysis was used to identify histologic patterns. Cox model was performed to estimate the hazard ratios associated with histologic parameters for kidney failure, defined as the need for long-term dialysis therapy of or eGFR <15 ml/min/1.73m2 at last follow-up. Multiplexed sequential Immunofluorescence and proximity ligation assay was used in kidney biopsies to analyze complement system activation. Results: Renal pathology in scleroderma renal crisis was more heterogeneous than expected, with 3 histological patterns of kidney injury identified by cluster analysis. Multivariable analysis showed that together with creatinine at presentation, acute arteriolar thrombotic microangiopathy and onion skinning in small arteries were independently associated with the risk of kidney failure. Multiplex immunofluorescence identified fractions from the complement classical pathway in arterioles and arteries in scleroderma renal crisis, while proximity ligation experiments confirmed the in situ activation of classical pathway C3 convertase. Complement terminal pathway fraction C5b-9 was localized in injured arteries. Conclusions: This study shows that the clinical definition of scleroderma renal crisis encompasses heterogeneity in the patterns of kidney injury. Acute arteriolar thrombotic microangiopathy and onion skinning were associated with kidney failure. Complement system was activated in these injured vessels
Introduction:Cryofibrinogen-associated nephropathy (CFN) is a very rare disease. Only few data are available about the clinicopathological presentation and treatment outcomes. Methods:Patients with cryofibrinogenemia (CF) diagnosed in French expert laboratories, and kidney biopsy findings suggestive of CFN (pauci-immune membranoproliferative glomerulonephritis [MPGN], thrombotic microangiopathy [TMA] and/or indirect signs of ischemia) were retrospectively included. Estimated glomerular filtration rate (eGFR), urinary protein-to-creatinine ratio (UPCR), and specific treatments were collected. Renal response (RR) was defined as a reduction of UPCR to < 0.5 g/g (or decrease > 50% if > 3 g/g at baseline) and an improvement of eGFR > 30% (if < 60 ml/min per 1.73 m2 and acute kidney injury (AKI) at baseline). Results:Among 2545 patients with CF, 232 (9%) underwent kidney biopsy, and only 28 (1%) had histological findings suggestive of CFN. Ten patients (36%) were female, and median age was 62 (interquartile range [IQR]: 49-71) years. eGFR at diagnosis was 21 (14-44) ml/min per 1.73 m2. Median UPCR was 3.30 (IQR: 1.52-4.85) g/g. AKI (78%) and nephrotic syndrome (41%) were frequent. Nine patients had an essential form, whereas 19 had a secondary form. MPGN was the most frequent pattern, with double contours (57%), nodular mesangial sclerosis (30%), mesangial (41%), endocapillary (56%) and extracapillary (11%) hypercellularity, and interstitial immune infiltration (41%). Thrombi were found in 43% of cases. Strikingly, 58% of the secondary forms were associated with a monoclonal gammopathy (MG). Patients with MG had more frequent skin manifestations (P = 0.002), endocapillary (P = 0.002), interstitial (P = 0.041) infiltration, and capillary thrombi (P = 0.012), and tended to have more frequent complement activation (P = 0.14). Sixty percent of patients were treated with various regimens of immunosuppressants (IS). After a mean follow-up of 476 (± 92) days, 65% of patients had an RR. Higher baseline eGFR (P = 0.04) and use of IS (P = 0.03) were predictive of RR. B-cell or plasma-cell depletion was effective in most cases associated with MG (80%). Conclusion:Our study, to the best of our knowledge, described for the first time the prevalence and the clinicopathological spectrum of CFN, which might be a very rare and underrecognized form of MG of renal significance presenting with pauci-immune MPGN ot TMA. IS are effective in most cases.
PURPOSE:To report on the oncological outcomes of active surveillance (AS) in low-grade prostate cancer (PCa) patients using the French SurACaP protocol, with a focus on long-term outcomes.METHODS:This multicenter study recruited patients with low-grade PCa between 2007 and 2013 in four referral centers in France. The cohort included patients meeting the SurACaP inclusion criteria, i.e., aged ≤75years, with low-grade PCa (i.e., ISUP 1), clinical stage T1c/T2a, PSA ≤10ng/mL and ≤3 positive cores and tumor length ≤3mm per core. The SurACaP protocol included a digital rectal examination every six months, PSA level measurement every three months for the first two years after inclusion and twice a year thereafter, a confirmatory biopsy in the first year after inclusion, and then follow-up biopsy every two years or if disease progression was suspected. Multiparametric magnetic resonance imaging (mpMRI) was progressively included over the study period.RESULTS:A total of 86 consecutive patients were included, with a median follow-up of 10.6 years. Only one patient developed metastases and died of PCa. The estimated rates of grade reclassification and treatment-free survival at 15 years were 53.4% and 21.2%, respectively. A negative mpMRI at baseline and a negative confirmatory biopsy were significantly associated with a lower risk of disease progression (P<0.05).CONCLUSIONS:AS using the French SurACaP protocol is a safe and valuable strategy for patients with low-risk PCa, with excellent oncological outcomes after more than 10 years' follow-up. Future studies are crucial to broaden the inclusion criteria and develop a personalized, risk based AS protocol with the aim of de-escalating follow-up examinations.LEVEL OF EVIDENCE:Grade 4.
Introduction Rapporter les résultats oncologiques de la surveillance active (SA) chez les patients atteints de cancer de la prostate (CP) de bas grade suivi selon le protocole français SurACaP, en mettant l’accent sur les résultats à long terme. Méthodes Il s’agit d’une étude multicentrique ayant recruté des patients atteints d’un CP de bas grade entre 2007 et 2013 dans quatre centres de référence en France. La cohorte comprenait des patients répondant aux critères d’inclusion de SurACaP, c’est-à-dire, âgés de≤75 ans, avec un CP de bas grade (c’est-à-dire, ISUP 1), un stade clinique T1c/T2a, un PSA≤10ng/mL et≤3 carottes positives et une longueur de tumeur≤3mm par carotte. Le protocole SurACaP comprenait un toucher rectal tous les six mois, une mesure du taux de PSA tous les trois mois pendant les deux premières années suivant l’inclusion et deux fois par an par la suite, une biopsie de confirmation la première année après l’inclusion, puis une biopsie de suivi tous les deux ans ou en cas de suspicion de progression de la maladie. L’imagerie par résonance magnétique multiparamétrique (IRMp) a été progressivement incluse au cours de la période d’étude. Résultats Au total, 86 patients consécutifs ont été inclus, avec un suivi médian de 10,6 ans. Un seul patient a développé des métastases et est décédé de son cancer de prostate. Les taux estimés de reclassification du grade et de survie sans traitement à 15 ans étaient respectivement de 53,4 % et de 21,2 %. Une IRMp négative au départ et une biopsie de confirmation négative étaient significativement associées à un risque plus faible de progression de la maladie (p<0,05). Conclusion La SA selon le protocole français SurACaP est une stratégie sûre et utile pour les patients atteints de CP à faible risque, avec d’excellents résultats oncologiques après plus de 10 ans de suivi. De futures études sont cruciales pour élargir les critères d’inclusion et développer un protocole de SA personnalisé, basé sur le risque, dans le but de désescalader les examens de suivi.
Abstract Background and Aims Most epidemiological studies in lupus nephritis (LN) report the risk of end-stage kidney disease (ESKD) (10-20% of patients at 10-15 years in developed countries), but less data is available on the long-term risk of chronic kidney disease (CKD), even though CKD is a major risk factor for morbidity and mortality. While immunosuppressive therapy for LN aims to reach and maintain remission in order to prevent CKD, it also exposes patients to cardiovascular, infectious and neoplastic adverse events. We conducted a retrospective cohort study to evaluate survival without CKD and adverse events in patients with LN. Method The MassiLUP cohort comprises all patients who underwent a kidney biopsy for a first flare of LN between 2001 and 2022 in the University Hospital of Marseille, France. We assessed patient survival, renal survival and survival without CKD (defined by an estimated glomerular filtration rate < 60 mL/min/1.73 m2), without cardiovascular event, without severe infection (requiring hospitalization, or zoster), and without cancer. Results A total of 168 patients (82.7% female; mean age 33 years; mean serum creatinine 102 µmol/L at the time of the first kidney biopsy) were included, with a mean follow-up of 9.6 ± 5.7 years. One (0.6%) patient had class I LN (ISN/RPS 2003 classification), 17 (10.1%) class II LN, 45 (26.8%) had class III (± V) LN, 73 (43.5%) had class IV (± V) LN, 29 (17.3%) patients had pure class V LN; 3 biopsies could not be classified. Four patients died during the follow-up (3 of severe infections and 1 of stroke); 41 (24.4%) patients developed CKD, among whom 19 (11.3%) patients reached ESKD; 34 (20.2%) patients presented a cardiovascular event, 59 (35.1%) a severe infection, and 12 (7.1%) a cancer. Survival rates without CKD were 82.9%, 74.6% and 67.3% at 5, 10 and 15 years, respectively. Survival rates without ESKD were 94.4%, 88.6% and 79.9% at 5, 10 and 15 years, respectively. Survival rates without cardiovascular event were 85.7%, 77.3% and 75.5% at 5, 10 and 15 years, respectively. Survival rates without severe infection were 76.3%, 63.8% and 51.8% at 5, 10 and 15 years, respectively. Survival rates without cancer were 96.3%, 92.9% and 85.6% at 5, 10 and 15 years, respectively. Conclusion Despite free access to treatment and care in France, the long-term prognosis of patients with lupus nephritis remains burdened by both a risk of CKD and ESKD, and a significant risk of cardiovascular and infectious events. This calls for continued efforts to improve therapeutic strategies, both in terms of efficacy and toxicity, and to strengthen the therapeutic alliance in the care of patients with LN.