Related Experiment Video
Updated: Aug 3, 2026
Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring
Published on: June 23, 2015
Changing Phenotypes and Clinical Outcomes Over Time in Microscopic Polyangiitis
Martina Uzzo1,2, Umberto Maggiore3, Filippo Sala1,4
1Department of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy.
Introduction:
Diagnosis and management of microscopic polyangiitis (MPA) have evolved considerably over the past decades, but it is unknown whether clinical and histological presentation and patient and renal outcomes have changed accordingly.
Methods:
We compared clinical and histopathological characteristic at diagnosis, risk of death, end-stage kidney disease (ESKD), and relapse rate in patients diagnosed with MPA between 1980 and 2022, after grouping them in 2 periods (p): p1980-2001 and p2002-2022. We compared the mortality rate between the 2 periods using Kaplan-Meier estimator and Cox-regression, and competing risks of ESKD and death using the Aalen-Johansen estimator, Fine-Gray multiple regression, and multistate models.
Results:
Out of 187 patients, 77 were in p1980-2001 and 110 in p2002 to 2022. Patients in p2002 to 2022 were older (66.2 ± 14.0 SD vs. 57.7 ± 15.8; P < 0.001), had a better kidney function (estimated glomerular filtration rate [eGFR] 25.9 ± 24.8 vs. 21.5 ± 28.2 ml/min per 1.73 m2; P = 0.011) and a lower prevalence of the Berden sclerotic class (5.9 vs. 20.9%; P = 0.011). Despite a similar crude and adjusted patient survival, the risk of ESKD decreased during p2002 to 2022 (subdistribution hazard ratio [HR] 0.30, 95% confidence interval [CI]: 0.16-0.57; P < 0.001). The results remained significant after accounting for death after ESKD and after adjusting for potential confounders (HR 0.33 [95% CI: 0.18-0.63; P < 0.001]). The risk of relapse was numerically higher during p2002 to 2022 (subdistribution-HR 1.64 [95% CI: 0.95-2.83; P = 0.075]).
Conclusion:
MPA kidney involvement has become less severe over the past decades, leading to a reduced risk of ESKD and a higher relapse rate, despite a comparable risk of death.
Insights
Microscopic polyangiitis (MPA) patients diagnosed more recently show less severe kidney disease and a reduced risk of end-stage kidney disease (ESKD). However, the risk of MPA relapse has increased, while the risk of death remains comparable.
Area of Science:
- Nephrology
- Rheumatology
- Internal Medicine
Background:
- Diagnosis and management of microscopic polyangiitis (MPA) have evolved.
- It remains unclear if clinical presentation, histological findings, and patient/renal outcomes have changed over time.
Purpose of the Study:
- To compare clinical and histopathological characteristics at diagnosis.
- To assess changes in the risk of death, end-stage kidney disease (ESKD), and relapse rate in MPA patients over time.
Main Methods:
- Retrospective analysis of 187 MPA patients diagnosed between 1980-2022, divided into two periods: 1980-2001 and 2002-2022.
- Comparison of clinical and histopathological features, mortality, ESKD risk, and relapse rates using statistical models including Kaplan-Meier, Cox-regression, Aalen-Johansen, and Fine-Gray regression.
Main Results:
- Patients diagnosed between 2002-2022 were older, had better kidney function (higher eGFR), and lower prevalence of severe sclerotic lesions compared to 1980-2001.
- The risk of ESKD significantly decreased in the later period (HR 0.30, P < 0.001), with results sustained after adjustments.
- Relapse risk was numerically higher in the 2002-2022 period (HR 1.64, P = 0.075), while patient survival was comparable between groups.
Conclusions:
- MPA kidney involvement has become less severe over recent decades.
- This has led to a reduced risk of ESKD but an increased relapse rate.
- The risk of death associated with MPA has remained comparable across the studied periods.
More Related Videos
05:14Author Spotlight: Overcoming Anti-VEGF Resistance Through Advanced Vascular Morphology Assessment in Choroidal Neovascularization
Published on: August 11, 2023
08:57Author Spotlight: Genetically Engineered Mouse Models and Pathological Characterization of Neurofibromatosis Type 1 Associated Tumors
Published on: May 17, 2024
