Clinical Presentation and Outcomes of Antineutrophil Cytoplasmic Autoantibody-Negative Pauci-Immune Glomerulonephritis
In a multicentre cohort of 259 patients with biopsy-proven pauci-immune glomerulonephritis, those without detectable ANCA presented younger, with worse kidney function and more proteinuria, and reached end-stage kidney disease more often at 1 and 3 years despite relapsing less. They also received standard induction and maintenance immunosuppression less often than ANCA-positive patients.
Background
ANCA-associated vasculitis is a rare, complex autoimmune condition. Although ANCAs have a pathogenic role, they are considered a suboptimal biomarker of disease activity, and previous work has suggested that patients without detectable circulating autoantibody differ in phenotype and outcome. This study set out to characterise presentation, histopathology, treatment practice, and outcomes in ANCA-negative pauci-immune glomerulonephritis.
Study design
A retrospective, multicentre cohort study conducted from 2002 to 2022, including patients with biopsy-proven pauci-immune glomerulonephritis. A total of 132 ANCA-negative and 127 ANCA-positive patients were included, with comparisons controlled for age, sex, ethnicity, and recruiting centre.
Key findings
ANCA-negative patients were younger (P < 0.001), more commonly presented with renal-limited disease (P < 0.001), had worse estimated glomerular filtration rate at diagnosis (P < 0.02), and higher rates of proteinuria (P < 0.01). After controlling for confounders, ANCA-negative patients had lower rates of relapse (P < 0.001) but higher rates of end-stage kidney disease at both 1 and 3 years (P < 0.001). Standard remission induction and maintenance therapies were used less often in the ANCA-negative group.
Clinical implications
The authors suggest adverse outcomes may result from delays in diagnosis, more advanced disease at presentation, and less intensive immunosuppressive treatment, and argue that ANCA-negative disease may represent a distinct entity. They conclude that current classification criteria inadequately address seronegative disease and that collaborative research including ANCA-negative patients in trials is needed.
Category
Research
Source
Kidney International Reports
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