Clinical Presentation and Outcomes of Antineutrophil Cytoplasmic Autoantibody-Negative Pauci-Immune Glomerulonephritis

被引：3

作者：

Floyd, Lauren ^{[1
,2
]}

Shetty, Anamay ^{[3
]}

Morris, Adam D. ^{[2
]}

Galesic, Kresimir ^{[4
]}

Elsayed, Mohamed ^{[2
]}

Lavery, Grace ^{[2
]}

Dhutia, Amrita ^{[5
]}

O'Brien, Sorcha ^{[6
]}

Stoneman, Sinead ^{[7
]}

Egan, Allyson ^{[8
]}

Little, Mark A. ^{[6
]}

Kratky, Vojtech ^{[9
,10
]}

Hruskova, Zdenka ^{[9
,10
]}

Tesar, Vladimir ^{[9
,10
]}

Kollar, Marek ^{[11
]}

von Bergwelt-Baildon, Anke ^{[12
]}

Schoenermarck, Ulf ^{[12
]}

Wu, Eveline Y. ^{[13
]}

Blazek, Lauren ^{[14
]}

Derebail, Vimal K. ^{[14
]}

Al-Attar, Mariam ^{[15
]}

Brown, Nina ^{[1
,15
]}

Alamo, Beatriz Sanchez ^{[16
]}

Leung, Wing-Yin ^{[17
]}

Chang, Bryan ^{[18
]}

Urban, Maria Letizia ^{[19
]}

Alberici, Federico ^{[20
]}

Quintana, Luis F. ^{[21
]}

Flossmann, Oliver ^{[22
]}

Brix, Silke R. ^{[23
]}

Geetha, Duvuru ^{[24
]}

McAdoo, Stephen ^{[5
]}

Dhaygude, Ajay P. ^{[1
,2
]}

Kronbichler, Andreas ^{[25
]}

Crnogorac, Matija ^{[4
]}

机构：

[1] Univ Manchester, Div Cardiovasc Sci, Manchester, England

[2] Lancashire Teaching Hosp, Royal Preston Hosp, Renal Dept, Preston, England

[3] Oxford Univ Hosp NHS Fdn Trust, Oxford, England

[4] Dubrava Clin Hosp, Dept Nephrol & Dialysis, Zagreb, Croatia

[5] Imperial Coll, Hammersmith Hosp, Renal & Transplant Ctr, London, England

[6] Trinity Coll Dublin, Trinity Translat Med Inst, Trinity Kidney Ctr, Dublin, Ireland

[7] Cork Univ Hosp, Dept Renal Med, Cork, Ireland

[8] Tallaght Univ Hosp, Trinity Hlth Kidney Ctr, Dublin, Ireland

[9] Charles Univ Prague, Gen Univ Hosp Prague, Dept Nephrol, Prague, Czech Republic

[10] Charles Univ Prague, Fac Med 1, Prague, Czech Republic

[11] Inst Clin & Expt Med, Dept Pathol, Prague, Czech Republic

[12] Ludwig Maximilians Univ Munchen, LMU Univ Hosp, Dept Med 4, Nephrol Div, Munich, Germany

[13] Univ North Carolina, Dept Pediat, Div Allergy Immunol & Rheumatol, Chapel Hill, NC USA

[14] Univ North Carolina, UNC Kidney Ctr, Dept Med, Div Nephrol & Hypertens, Chapel Hill, NC USA

[15] Salford Royal Hosp, Northern Care Alliance NHS Fdn Trust, Renal Dept, Salford, England

[16] Hosp Univ Ramon & Cajal, Dept Nephrol, Madrid, Spain

[17] Manchester Univ NHS Fdn Trust, Renal Transplantat & Urol Unit, Manchester, England

[18] Univ Cambridge, Dept Med, Cambridge, England

[19] Univ Firenze, Dept Expt & Clin Med, Florence, Italy

[20] Univ Brescia, Dept Med & Surg Specialties, Brescia, Italy

[21] Univ Barcelona, Hosp Clin Barcelona, Dept Nephrol & Renal Transplantat, IDIBAPS, Barcelona, Spain

[22] Royal Berkshire Hosp, Dept Nephrol, Reading, Berks, England

[23] Univ Manchester, Div Cell Matrix Biol & Regenerat Med, Manchester, England

[24] Johns Hopkins Univ, Div Nephrol, Sch Med, Baltimore, MD USA

[25] Med Univ Innsbruck, Dept Internal Med Nephrol & Hypertens, Innsbruck, Austria

来源：

KIDNEY INTERNATIONAL REPORTS | 2025年 / 10卷 / 05期

基金：

爱尔兰科学基金会;

关键词：

ANCA-associated vasculitis; ANCA-negative; end-stage kidney disease; kidney biopsy; pauci-immune glomerulonephritis; vasculitis; RHEUMATOLOGY CLASSIFICATION CRITERIA; 2022; AMERICAN-COLLEGE; VASCULITIS; GRANULOMATOSIS; ASSOCIATIONS; ALLIANCE; MYELOPEROXIDASE; HISTOLOGY; DISEASE;

D O I：

10.1016/j.ekir.2025.02.032

中图分类号：

R5 [内科学]; R69 [泌尿科学（泌尿生殖系疾病）];

学科分类号：

1002 ; 100201 ;

摘要：

Introduction: Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is a rare, complex autoimmune condition. Although ANCAs have a pathogenic role, they are considered a suboptimal biomarker of disease activity. Previous studies suggest differences in clinical phenotypes and outcomes in those without detectable circulating autoantibody. This study aimed to investigate the clinical presentation, histopathological findings, treatment practices, and outcomes of patients with ANCA-negative pauciimmune glomerulonephritis (PIGN). Methods: A retrospective, multicenter cohort study was conducted from 2002 to 2022 and included those with biopsy-proven PIGN. We aimed to investigate differences in presentation, clinical outcomes, and treatment practices of patients with ANCA-negative PIGN when compared with ANCA-positive controls. Results: In total, 132 ANCA-negative and 127 ANCA-positive patients were included. 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). Controlling for age, sex, ethnicity, and recruiting center, ANCA-negative patients had lower rates of relapse (P < 0.001) and higher rates of end-stage kidney disease (ESKD) at 1 and 3 years (P < 0.001). Standard remission induction and maintenance therapies were used less often in ANCA-negative patients. Conclusion: The precise pathophysiology and factors contributing to the clinical phenotype of ANCAnegative PIGN remain unclear and potentially represent a distinct disease entity. Adverse outcomes may result from delays in diagnosis, advanced disease at presentation, and less intense immunosuppressive treatment. Current classification criteria inadequately address ANCA-negative disease and collaborative research, which includes ANCA-negative patients in trials is needed.

引用

页码：1450 / 1459

页数：10

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