SIGNIFICANCE STATEMENT: Reliable prediction tools are needed to personalize treatment in ANCA-associated GN. More than 1500 patients were collated in an international longitudinal study to revise the ANCA kidney risk score. The score showed satisfactory performance, mimicking the original study (Harrell's C=0.779). In the development cohort of 959 patients, no additional parameters aiding the tool were detected, but replacing the GFR with creatinine identified an additional cutoff. The parameter interstitial fibrosis and tubular atrophy was modified to allow wider access, risk points were reweighted, and a fourth risk group was created, improving predictive ability (C=0.831). In the validation, the new model performed similarly well with excellent calibration and discrimination ( n =480, C=0.821). The revised score optimizes prognostication for clinical practice and trials. BACKGROUND: Reliable prediction tools are needed to personalize treatment in ANCA-associated GN. A retrospective international longitudinal cohort was collated to revise the ANCA renal risk score. METHODS: The primary end point was ESKD with patients censored at last follow-up. Cox proportional hazards were used to reweight risk factors. Kaplan-Meier curves, Harrell's C statistic, receiver operating characteristics, and calibration plots were used to assess model performance. RESULTS: Of 1591 patients, 1439 were included in the final analyses, 2:1 randomly allocated per center to development and validation cohorts (52% male, median age 64 years). In the development cohort ( n =959), the ANCA renal risk score was validated and calibrated, and parameters were reinvestigated modifying interstitial fibrosis and tubular atrophy allowing semiquantitative reporting. An additional cutoff for kidney function (K) was identified, and serum creatinine replaced GFR (K0: <250 μ mol/L=0, K1: 250-450 μ mol/L=4, K2: >450 μ mol/L=11 points). The risk points for the percentage of normal glomeruli (N) and interstitial fibrosis and tubular atrophy (T) were reweighted (N0: >25%=0, N1: 10%-25%=4, N2: <10%=7, T0: none/mild or <25%=0, T1: ≥ mild-moderate or ≥25%=3 points), and four risk groups created: low (0-4 points), moderate (5-11), high (12-18), and very high (21). Discrimination was C=0.831, and the 3-year kidney survival was 96%, 79%, 54%, and 19%, respectively. The revised score performed similarly well in the validation cohort with excellent calibration and discrimination ( n =480, C=0.821). CONCLUSIONS: The updated score optimizes clinicopathologic prognostication for clinical practice and trials.
- MeSH
- ANCA-asociované vaskulitidy * diagnóza MeSH
- atrofie MeSH
- fibróza MeSH
- kreatinin MeSH
- ledviny MeSH
- lidé středního věku MeSH
- lidé MeSH
- longitudinální studie MeSH
- protilátky proti cytoplazmě neutrofilů * MeSH
- retrospektivní studie MeSH
- rizikové faktory MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
The N-PATH (Nephrology Partnership for Advancing Technology in Healthcare) program concluded with the 60th European Renal Association 2023 Congress in Milan, Italy. This collaborative initiative aimed to provide advanced training in interventional nephrology to young European nephrologists. Funded by Erasmus+ Knowledge Alliance, N-PATH addressed the global burden of chronic kidney disease (CKD) and the shortage of nephrologists. CKD affects >850 million people worldwide, yet nephrology struggles to attract medical talent, leading to unfilled positions in residency programs. To address this, N-PATH focused on enhancing nephrology education through four specialized modules: renal expert in renal pathology (ReMAP), renal expert in vascular access (ReVAC), renal expert in medical ultrasound (ReMUS) and renal expert in peritoneal dialysis (RePED). ReMAP emphasized the importance of kidney biopsy in nephrology diagnosis and treatment, providing theoretical knowledge and hands-on training. ReVAC centred on vascular access in haemodialysis, teaching trainees about different access types, placement techniques and managing complications. ReMUS recognized the significance of ultrasound in nephrology, promoting interdisciplinary collaboration and preparing nephrologists for comprehensive patient care. RePED addressed chronic peritoneal dialysis, offering comprehensive training in patient selection, prescription, monitoring, complications and surgical techniques for catheter insertion. Overall, N-PATH's strategy involved collaborative networks, hands-on training, mentorship, an interdisciplinary approach and the integration of emerging technologies. By bridging the gap between theoretical knowledge and practical skills, N-PATH aimed to revitalize interest in nephrology and prepare proficient nephrologists to tackle the challenges of kidney diseases. In conclusion, the N-PATH program aimed to address the shortage of nephrologists and improve the quality of nephrology care in Europe. By providing specialized training, fostering collaboration and promoting patient-centred care, N-PATH aimed to inspire future nephrology professionals to meet the growing healthcare demands related to kidney diseases and elevate the specialty's status within the medical community.
ANCA-asociované vaskulitidy jsou nekrotizující vaskulitidy malých cév s minimem imunodepozit. Obvykle jsou doprovázené pozitivitou ANCA protilátek (AntiNeutrophil Cytoplasmic Antibody, protilátky proti cytoplazmě neutrofilů), jejichž cílovými antigeny může být proteináza 3 (PR3-ANCA) nebo myeloperoxidáza (MPO-ANCA). Mezi ANCA-asociované vaskulitidy patří granulomatóza s polyangiitidou (dříve Wegenerova), mikroskopická polyangiitida a eozinofilní granulomatóza s polyangiitidou (dříve syndrom Churga a Straussové). Nejčastěji postiženými orgány bývají plíce a dýchací cesty, ORL oblast a ledviny. Při postižení ledvin je typickým projevem pauciimunní nekrotizující srpkovitá rychle progredující glomerulonefritida. Akutním život ohrožujícím stavem je pulmo-renální syndrom s krvácením do plic a selháváním ledvin. V diagnostice se využívá stanovení ANCA protilátek, zobrazovací metody a biopsie. Pro dobrou prognózu je nezbytné časné stanovení správné diagnózy i časné podání adekvátní terapie, kterou dnes nejčastěji bývá kombinace kortikosteroidů a buď cyklofosfamidu nebo rituximabu (monoklonální protilátky proti antigenu CD20). Stále je možné zvážit v léčbě těžkých případů přidání plazmaferézy. Rituximab je lékem volby v terapii relabující vaskulitidy. Adresa pro korespondenci: MUDr. Zdenka Hrušková, PhD. Klinika nefrologie VFN a 1. LF UK U Nemocnice 499/2 128 08 Praha 2 email: hruskova.zdenka@vfn.cz
ANCA-associated vasculitides (AAV) are small-vessel necrotizing vasculitides, with no or few immune deposits. They are usually associated with the presence of ANCA antibodies (AntiNeutrophil Cytoplasmic Antibody), targeted either against proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA). ANCA-associated vasculitides include granulomatosis with polyangiitis (formerly Wegener‘s), microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome). The most commonly afflicted organs involve the lungs and the respiratory tract, ENT area, and the kidneys. Renal involvement typically manifests as pauci-immune necrotizing crescentic rapidly progressive glomerulonpehritis. Pulmo-renal syndrome with lung haemorrhage and deteriorating kidney function may be acutely life-threatening. Diagnostic methods include ANCA measurement, imaging methods and biopsy. Early recognition of the diagnosis and an early start of adequate treatment are necessary for a good outcome. The current treatment typically consists of corticosteroids and either cyclophoshapmide or rituximab (a monoclonal antibody directed against CD20 antigen). The addition of plasma exchange may be considered in severe cases. Rituximab is preferred for the treatment of all relapsing forms of this vasculitis.
INTRODUCTION: Acute tubulointerstitial nephritis (ATIN) is a well-recognized cause of acute kidney injury (AKI) due to the tubulointerstitial inflammation. The aim of this study was to explore the clinical features, outcomes, and responses to corticosteroid treatment in patients with ATIN. METHODS: Patients with biopsy-proven ATIN, who were diagnosed between 1994 and 2016 at the Department of Nephrology, Charles University, First Faculty of Medicine, and General University Hospital in Prague, were included in the study. Patient demographics, the aetiological and clinical features, the treatment given, and the outcome at 1 year of follow-up were extracted from patient records. RESULTS: A total of 103 ATIN patients were analysed, of which 68 had been treated with corticosteroids. There was no significant difference in the median serum creatinine 280 (169-569) μmol/L in the conservatively managed group versus 374 (249-558) μmol/L in the corticosteroid-treated group, p = 0.18, and dependence on dialysis treatment at baseline at the time of biopsy (10.3 vs. 8.6%). During the 1 year of follow-up, those ATIN patients who had been treated with corticosteroids did better and showed greater improvement in kidney function, determined as serum creatinine difference from baseline and from 1 month over 1-year period (p = 0.001). CONCLUSIONS: This single-centre retrospective cohort study supports the beneficial role of the administration of corticosteroid therapy in the management of ATIN.
- MeSH
- dialýza ledvin * škodlivé účinky MeSH
- hormony kůry nadledvin terapeutické užití MeSH
- intersticiální nefritida * farmakoterapie diagnóza MeSH
- kreatinin MeSH
- ledviny patologie MeSH
- lidé MeSH
- retrospektivní studie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- Geografické názvy
- Česká republika MeSH
- MeSH
- biologické markery MeSH
- budesonid škodlivé účinky MeSH
- glomerulus patologie MeSH
- IgA nefropatie * diagnóza farmakoterapie patologie MeSH
- imunoglobulin A MeSH
- lidé MeSH
- proteinurie patologie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
Doporučené postupy pro praktické lékaře Doporučené diagnostické a terapeutické postupy pro všeobecné praktické lékaře
29 stran ; 30 cm
Brožura a směrnice, které se zaměřují na diagnostiku a léčbu nemocí ledvin. Určeno praktickým lékařům.
- MeSH
- nemoci ledvin diagnóza terapie MeSH
- praktické lékařství MeSH
- Publikační typ
- směrnice pro lékařskou praxi MeSH
- Konspekt
- Patologie. Klinická medicína
- NLK Obory
- nefrologie
- všeobecné lékařství
- NLK Publikační typ
- brožury
