ArticleClinical rheumatology2024
Exostosin-1/exostosin-2 expression and favorable kidney outcomes in lupus nephritis: a retrospective cohort study.
Article in Clinical rheumatology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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Who cites it
6 citing papers in PubMed.
- Long-term kidney and patient outcomes in pure versus mixed lupus membranous nephritis.Clinical kidney journal · 2026Article
- Detection of Exostosin 1 in Lupus Nephritis: Prevalence, Clinico-Pathologic and Renal Outcome Correlations.Diagnostics (Basel, Switzerland) · 2026Article
- Not So Benign: Revisiting Pure Membranous Lupus Nephritis.Journal of personalized medicine · 2025Review
- Exostosin 1/exostosin 2-associated membranous nephropathy in undifferentiated connective tissue disease: a case report and literature review.BMC nephrology · 2025Review
- Antigens in membranous nephropathy: discovery and clinical implications.Nature reviews. Nephrology · 2025Review
- Repeat biopsies in membranous lupus nephritis in the era of target antigen identification.Virchows Archiv : an international journal of pathology · 2025Article
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Abstract
INTRODUCTION/
objectivesThe heterodimer exostosin-1/exostosin-2 (EXO-1/2) is a novel antigen observed in membranous nephropathy associated with systemic lupus erythematosus. This study aimed to evaluate the association between EXO-1/2 positivity in kidney biopsy and kidney outcomes.
methodsThe kidney biopsy tissue from 50 class 5 lupus nephritis (LN) and 55 mixed class 3/4 + 5 LN patients was stained for EXO-1/2. Baseline clinical and histological characteristics were compared between EXO-1/2 positive and EXO-1/2 negative patients. Time-to-event analyses were performed to compare rates of response to therapy, kidney flares, and progression to a 40% decline of the glomerular filtration rate (eGFR), doubling of serum creatinine, and kidney failure.
resultsFourteen out of 50 (28%) of class 5 and 5 out of 55 (9%) of mixed class 3/4 + 5 LN stained positive for EXO-1/2. Patients with class 5 LN and EXO-1/2 positive stain were younger, with better kidney function at presentation, and lower scarring in the kidney biopsy analysis. Over a median follow-up of 100 months, patients with positive EXO-1/2 staining had significantly lower rates of progression in the full cohort. When analyzed separately in class 5 and mixed class LN subgroups, there were significantly lower rates of progression to a 40% decline of the eGFR and non-statistically significant trends for doubling of serum creatinine and kidney failure.
conclusionEXO-1/2 is a novel antigen detected in class 5 LN and associated with a good prognosis of kidney function. The incorporation of EXO-1/2 staining in clinical practice can potentially modify the management of LN due to its prognostic implications. Key Points • Exostosin-1/exostosin-2 antigen has been found in cases of membranous nephropathy associated with autoimmune diseases such as systemic lupus erythematosus. • Exostosin-1/exostosin-2 staining in the kidney biopsy of class 5 or mixed class 3/4 + 5 lupus nephritis is associated with a good long-term prognosis of kidney function. • The incorporation of exostosin-1/exostosin-2 staining into clinical practice can potentially modify management due to its prognostic implications.
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