Kidney Outcomes in Autoimmune Blistering Diseases: Comparative Real-World Cohort Study of Bullous Pemphigoid and Pemphigus Vulgaris

Background: Bullous pemphigoid (BP) and pemphigus vulgaris (PV) are autoimmune blistering skin disorders that may share underlying immunologic mechanisms with glomerular disease. Although membranous nephropathy has been reported in BP, population-based data on kidney outcomes in these conditions are limited. Methods: Using the TriNetX Global Collaborative Network, we conducted a retrospective cohort study across 147 healthcare organizations. We identified adults (≥18 years) with a new diagnosis of BP (ICD-10: L12.0) or PV (L10.0) between 2005 and 2025. Patients with pre-existing kidney disease including glomerular disease, CKD, dialysis, or kidney transplant, were excluded. Renal outcomes were assessed 12–24 months after diagnosis. Primary outcomes included new diagnoses of chronic kidney disease (CKD; N18.x), acute kidney injury (AKI; N17), glomerulonephritis (GN; N00–N08), hematuria (R31), and proteinuria (R80). Secondary outcomes included dialysis, kidney biopsy, and all-cause mortality. Kaplan-Meier analysis was used to evaluate renal event-free survival. Results: The BP cohort (n = 29,801) had higher incidence rates of CKD (6.8% vs 3.0%), AKI (3.3% vs 1.7%), and hematuria (1.5% vs 0.1%) compared to the PV cohort (n = 10,801). Proteinuria rates were similar (0.6% vs 0.5%). GN was identified in 0.06% of BP patients and not observed in PV. No dialysis or kidney biopsies were recorded in either group. All-cause mortality was higher in BP (4.2% vs 1.5%). Kaplan-Meier curves showed reduced renal event-free survival in BP patients, particularly for CKD and AKI (CKD-free survival: BP 87.8% vs PV 95.6%). Conclusion: This real-world analysis suggests that patients with bullous pemphigoid may be at increased risk for adverse kidney outcomes, including CKD, AKI, and hematuria, compared to those with pemphigus vulgaris. While causality cannot be inferred, these findings highlight a potential renal association in BP and support the need for closer kidney function monitoring in patients with autoimmune blistering skin diseases.

Authors

Institutions

Publication Details

Journal
Journal of the American Society of Nephrology
Published
2025-10-01
DOI
https://doi.org/10.1681/asn.2025gd190j80
Primary Topic
Autoimmune Bullous Skin Diseases
Type
article
Field-Weighted Citation Impact
0.00
Controls
|||
ALL TIME
JAN
FEB
MAR
APR
MAY
JUN
JUL
AUG
SEP
OCT
article

Kidney Outcomes in Autoimmune Blistering Diseases: Comparative Real-World Cohort Study of Bullous Pemphigoid and Pemphigus Vulgaris

Nasrollah Ghahramani, Amanda Karasinski, Bayan Matarneh, Fareeha Khalil et al.
Journal of the American Society of Nephrology
Autoimmune Bullous Skin Diseases
article

Kidney Outcomes in Autoimmune Blistering Diseases: Comparative Real-World Cohort Study of Bullous Pemphigoid and Pemphigus Vulgaris

Nasrollah Ghahramani, Amanda Karasinski, Bayan Matarneh, Fareeha Khalil, Naman Trivedi, Ahmad Matarneh, Umar Farooq
article en

Abstract

Background: Bullous pemphigoid (BP) and pemphigus vulgaris (PV) are autoimmune blistering skin disorders that may share underlying immunologic mechanisms with glomerular disease. Although membranous nephropathy has been reported in BP, population-based data on kidney outcomes in these conditions are limited. Methods: Using the TriNetX Global Collaborative Network, we conducted a retrospective cohort study across 147 healthcare organizations. We identified adults (≥18 years) with a new diagnosis of BP (ICD-10: L12.0) or PV (L10.0) between 2005 and 2025. Patients with pre-existing kidney disease including glomerular disease, CKD, dialysis, or kidney transplant, were excluded. Renal outcomes were assessed 12–24 months after diagnosis. Primary outcomes included new diagnoses of chronic kidney disease (CKD; N18.x), acute kidney injury (AKI; N17), glomerulonephritis (GN; N00–N08), hematuria (R31), and proteinuria (R80). Secondary outcomes included dialysis, kidney biopsy, and all-cause mortality. Kaplan-Meier analysis was used to evaluate renal event-free survival. Results: The BP cohort (n = 29,801) had higher incidence rates of CKD (6.8% vs 3.0%), AKI (3.3% vs 1.7%), and hematuria (1.5% vs 0.1%) compared to the PV cohort (n = 10,801). Proteinuria rates were similar (0.6% vs 0.5%). GN was identified in 0.06% of BP patients and not observed in PV. No dialysis or kidney biopsies were recorded in either group. All-cause mortality was higher in BP (4.2% vs 1.5%). Kaplan-Meier curves showed reduced renal event-free survival in BP patients, particularly for CKD and AKI (CKD-free survival: BP 87.8% vs PV 95.6%). Conclusion: This real-world analysis suggests that patients with bullous pemphigoid may be at increased risk for adverse kidney outcomes, including CKD, AKI, and hematuria, compared to those with pemphigus vulgaris. While causality cannot be inferred, these findings highlight a potential renal association in BP and support the need for closer kidney function monitoring in patients with autoimmune blistering skin diseases.

Journal of the American Society of NephrologyVol. 36(10S)
Penn State Milton S. Hershey Medical Center (US)
Openalex Percentile: Top 57%
Autoimmune Bullous Skin Diseases
AI Navigator

Ask Laika to Summarize, Analyze, and Connect papers live on the map.

Summarize Papers & Methodologies

Extract key findings, datasets, and comparative methods across publications.

Benchmark Rankings & Visual Analytics

Rank top research institutions, authors, funders, topics, and journals by Field-Weighted Citation Impact (FWCI) and paper volume with instant charts.

Connect Distant Disciplines

Bridge topological clusters on the map to find hidden collaborative intersections.