AB0500 CLINICAL CHARACTERISTICS AND POTENTIAL BIOMARKERS FOR DISEASE ACTIVITY OF PATIENTS WITH ANCA ASSOCIATED VASCULITIS: A MONOCENTER STUDY IN CHINA

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1548-1548
Author(s):  
Y. Liu ◽  
L. MA ◽  
L. Jiang
Keyword(s):  
Disease Activity ◽  
Granulomatosis With Polyangiitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Chinese Patients ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
American College Of Rheumatology ◽  
Chapel Hill ◽  
Anca Associated Vasculitis ◽  
Incident Cases

Background:Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are a group of multisystem, autoimmune, inflammatory disease characterized by pauci- necrotizing vasculitis affecting small blood vessels. The clinical manifestations of the AAV are diverse and can be confined to one organ, or multiple organs and even life-threatening. However, there has been no specific index for assessing the activity of AAV at diagnosis.Objectives:The aim of this study was to describe the clinical and serological features of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in eastern China using data from a hospital-based study. And looking for indicators that can predict disease activity.Methods:We retrospectively studied patients with newly diagnosed AAV evaluated from January 1, 2009, to December 31, 2018. In total, 219 patients diagnosed were classified according to the American College of Rheumatology classification criteria and/or revised Chapel Hill 2012 definitions, and their clinical and serological features were evaluated. The association of laboratory data with disease activity was assessed via regression models.Results:Of 219 incident cases of AAV, 37/219 (16.9%) had granulomatosis with polyangiitis (GPA), 172/219 (78.5%) were microscopic polyangiitis (MPA), and 10/219 (4.6%) had eosinophilic granulomatosis with polyangiitis (EGPA). The mean age at diagnosis of patients with GPA were 51.5 years MPA were 61.7 years, and EGPA were 49.8 years, respectively. Patients with MPA were significantly older than GPA and EGPA at diagnosis (p<0.001). ANCAs tested positive in 207 (94.5%) of cases: 167 (80.7%) were MPO-ANCA and 40 (19.3%) were PR3-ANCA. Lung, skin, nervous system symptoms were the most common in EGPA. For GPA, ear–nose–throat (ENT) symptoms and lungs involvement were the most common. Renal and lung involvement occurs most frequently in MPA. In the multivariable logistic regression analysis, higher anti-MPO antibody (149.4 IU/ml), higher hypersensitive c-reactive protein (hs-CRP, 62.5 mg/L), lower hemoglobin (113.5g/L), and higher complement 4 (C4, >0.215 g/L) were proved to be independent risk factors for active disease. Further research showed that C4 had higher sensitivity (70.0%) and specificity (83.4%) than the other three indicators.Conclusion:MPO-ANCA-positive MPA is the most common form of AAV in Chinese patients. Serum C4 concentrations at diagnosis might be a useful biomarker of disease activity in AAV.References:[1]Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides.Arthritis Rheum2013, 65:1-11.[2]Choi H, Kim Y, Jung SM, Song JJ, Park Y-B, Lee S-W. Low serum complement 3 level is associated with severe ANCA-associated vasculitis at diagnosis.Clinical and Experimental Nephrology2018, 23:223-230.[3]Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis.Arthritis Rheum1990, 33:1101-1107.[4]Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis).Arthritis Rheum1990, 33:1094-1100.[5]Mukhtyar C, Lee R, Brown D, Carruthers D, Dasgupta B, Dubey S, et al. Modification and validation of the Birmingham Vasculitis Activity Score (version 3).Ann Rheum Dis2009, 68:1827-1832.[6]Markiewski MM, Lambris JD. The role of complement in inflammatory diseases from behind the scenes into the spotlight.Am J Pathol2007, 171:715-727.Disclosure of Interests:None declared

scholarly journals Clinical characteristics and potential biomarkers for disease activity of patients with ANCA associated vasculitis: a monocenter study in China

2020 ◽  
Author(s):  
Yun Liu ◽  
Lili Ma ◽  
Zongfei Ji ◽  
Rongyi Chen ◽  
Xiufang Kong ◽  
...  
Keyword(s):  
Disease Activity ◽  
Granulomatosis With Polyangiitis ◽  
Eastern China ◽  
Laboratory Data ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Chinese Patients ◽  
Multivariable Logistic Regression Analysis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Anca Associated Vasculitis ◽  
Incident Cases

Abstract Objective: The aim of this study was to describe the clinical and serological features of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in eastern China using data from a hospital-based study. And looking for indicators that can predict disease activity. Methods: We retrospectively studied patients with newly diagnosed AAV evaluated from January 1, 2009, to December 31, 2018 . In total, 219 patients diagnosed were classified according to the American College of Rheumatology classification criteria and/or revised Chapel Hill 2012 definitions, and their clinical and serological features were evaluated. The association of laboratory data with disease activity was assessed via regression models. Results: Of 219 incident cases of AAV, 37/219 (16.9%) had granulomatosis with polyangiitis (GPA), 172/219 (78.5%) were microscopic polyangiitis (MPA), and 10/219 (4.6%) had eosinophilic granulomatosis with polyangiitis (EGPA). The mean age at diagnosis of patients with GPA were 51.5 years (male/female, 18/19), MPA were 61. 7 years (male/female, 84/88), and EGPA were 49.8 years ( male/female, 7/3), respectively. Patients with MPA were significantly older than GPA and EGPA at diagnosis ( p <0.001). ANCAs tested positive in 207 (94.5%) of cases: 167 (80.7%) were MPO-ANCA and 40 (19.3%) were PR3-ANCA. Lung, skin, nervous system symptoms were the most common in EGPA. For GPA, ear–nose–throat (ENT) symptoms and lungs involvement were the most common. Renal and lung involvement occurs most frequently in MPA. In the multivariable logistic regression analysis, higher anti-MPO antibody ( 149.4 IU/ml), higher hypersensitive c-reactive protein (hs-CRP, 62.5 mg/L), lower hemoglobin ( 113.5g/L), and higher complement 4 (C4, >0.215 g/L) were proved to be independent risk factors for active disease. Further research showed that C4 had higher sensitivity (70.0%) and specificity (83.4%) than the other three indicators. Conclusion: MPO-ANCA-positive MPA is the most common form of AAV in Chinese patients. Serum C4 concentrations at diagnosis might be a useful biomarker of disease activity in AAV.

scholarly journals Proposal for a more practical classification of antineutrophil cytoplasmic antibody-associated vasculitis

2020 ◽  
Author(s):  
Nestor Oliva-Damaso ◽  
Andrew S Bomback
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Clinical Information ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Consensus Conference ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Chapel Hill ◽  
Chapel Hill Consensus Conference ◽  
Eosinophilic Granulomatosis ◽  
Relapse Rates

Abstract The nomenclature for antineutrophil cytoplasmic antibody (ANCA)-associated kidney disease has evolved from honorific eponyms to a descriptive-based classification scheme (Chapel Hill Consensus Conference 2012). Microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis do not correlate with presentation, response rates and relapse rates as when comparing myeloperoxidase versus leukocyte proteinase 3. Here we discuss the limitations of the currently used classification and propose an alternative, simple classification according to (i) ANCA type and (ii) organ involvement, which provides important clinical information of prognosis and outcomes.

scholarly journals ANCA Status or Clinical Phenotype — What Counts More?

2021 ◽  
Vol 23 (6) ◽  
Author(s):  
Martin Windpessl ◽  
Erica L. Bettac ◽  
Philipp Gauckler ◽  
Jae Il Shin ◽  
Duvuru Geetha ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Granulomatosis With Polyangiitis ◽  
Clinical Phenotype ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Cardiovascular Death ◽  
Genetic Associations ◽  
Ongoing Debate ◽  
The Past ◽  
Anca Associated Vasculitis

Abstract Purpose of Review There is ongoing debate concerning the classification of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. That is, whether classification should be based on the serotype (proteinase 3 (PR3)- or myeloperoxidase (MPO)-ANCA) or on the clinical phenotype (granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA)). To add clarity, this review focused on integration of the most recent literature. Recent Findings Large clinical trials have provided evidence that a serology-based risk assessment for relapses is more predictive than distinction based on the phenotype. Research conducted in the past decade indicated that a serology-based approach more closely resembles the genetic associations, the clinical presentation (i.e., lung involvement), biomarker biology, treatment response, and is also predicting comorbidities (such as cardiovascular death). Summary Our review highlights that a serology-based approach could replace a phenotype-based approach to classify ANCA-associated vasculitides. In future, clinical trials and observational studies will presumably focus on this distinction and, as such, translate into a “personalized medicine.”

scholarly journals POS0830 FACTORS INFLUENCING PATIENT-REPORTED OUTCOMES IN ANCA-ASSOCIATED VASCULITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 668.2-669
Author(s):  
S. Monti ◽  
P. Delvino ◽  
C. Klersy ◽  
G. Coppa ◽  
A. Milanesi ◽  
...  
Keyword(s):  
Disease Activity ◽  
Global Assessment ◽  
Patient Reported Outcomes ◽  
Granulomatosis With Polyangiitis ◽  
Disease Duration ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Anca Associated Vasculitis ◽  
Organ Systems ◽  
Damage Accrual ◽  
Patient Reported

Background:Patient-reported outcomes (PROs) are currently poorly integrated in the clinical evaluation of disease activity in patients with ANCA-associated vasculitis (AAV).Objectives:To assess the distribution of the Patient Global Assessment (PtGA) in patients with AAV in stable remission, and to identify correlates of PtGA; to assess the discordance between PtGA score and PhGA.Methods:Patients with a diagnosis of AAV [eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis, microscopic polyangiitis] in stable, complete remission (defined by a BVAS=0) and with a Physician Global Assessment (PhGA)=0 were included. A questionnaire including several aspects of disease captured by PROs was collected. PtGA on a 0-100 mm visual analogue scale (VAS) was assessed, with higher scores representing higher/worse levels of disease activity. Similarly, VAS for pain, chronic damage according to the patient’s opinion, general health (GH), fatigue, and sleep quality were collected. The worst symptom in the patient’s opinion affecting the overall assessment of disease activity was recorded. The Cragg Hurdle model was used to assess the predictors of PtGA.Results:65 patients were included, female 57%, mean age 61±12 years. Mean disease duration at enrollment was 8±6 years. Mean vasculitis damage index (VDI) was 4.4 ±2.3, with 45% of patients having a VDI ≥ 5. Despite having been classified as being in remission, PtGA was elevated in 37% of patients. We explored several correlates of PtGA. Higher degree of damage accrual (VDI) did not influence the patient’s evaluation of current disease activity. Similarly, we did not identify a correlation between older age, educational level, number of organ-systems involved, number of comorbidities, the number of previous major or minor relapses, higher disease duration, nor the type of AAV diagnosis (figure 1, panel A). Only sex significantly correlated with PtGA scores: 19 (51%) of female patients reported an elevated PtGA compared to only 5 (18%) of male (p=0.009). PtGA resulted to be significantly correlated with other (mostly modifiable) PROs including VAS pain, perception of the level of chronic damage accrual, GH, and fatigue (figure 1, panel B). The agreement between patients’ and physicians’ assessments of disease activity was 63%. Patients reported pain, followed by chronic respiratory symptoms to be the worst-experienced ongoing manifestations affecting their evaluation of disease activity.Conclusion:A significant proportion of patients with AAV considered to be in remission by the physician still declares to have persistent aspects of uncontrolled disease. PtGA is significantly influenced by persistent pain and fatigue, which warrant better assessment in the future.Disclosure of Interests:None declared

scholarly journals A Rare Case of Digital Ischemia and Gangrene in ANCA-Associated Vasculitis with Review of the Literature

2017 ◽  
Vol 2017 ◽  
pp. 1-7 ◽  
Author(s):  
Richard A. Lau ◽  
Ramandeep Bains ◽  
Duminda Suraweera ◽  
Jane Ma ◽  
Emil R. Heinze ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
English Literature ◽  
Renal Involvement ◽  
Antineutrophil Cytoplasmic Antibody ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Proteinase 3 ◽  
Anca Associated Vasculitis ◽  
Digital Ischemia

This paper describes one patient with Antineutrophil Cytoplasmic Antibody- (ANCA-) associated vasculitis who initially presented with multiple ischemic fingers and toes. On further evaluation, the patient was also found to have pulmonary-renal involvement and episcleritis. The diagnosis was supported with a positive cANCA (anti-proteinase 3) and a bronchoscopy consistent with diffuse alveolar hemorrhage. Although the patient refused a tissue biopsy, clinical presentation including nasal ulceration, sinus congestion, and epistaxis and anti-proteinase 3 antibody were more consistent with Granulomatosis with Polyangiitis (GPA) rather than Microscopic Polyangiitis (MPA) or Eosinophilic Granulomatosis with Polyangiitis (EGPA) based on the recently presented ACR/EULAR Provisional 2017 Classification Criteria for GPA (Luqmani et al., 2016). The patient responded well to therapy including high dose steroids and cyclophosphamide, with improvement of all organs involved and had no further digital ischemia or gangrene on follow-up. We include a review of the English literature summarizing presentation, management, and outcome of 16 similar cases.

Association of venous thromboembolic events with skin, pulmonary and kidney involvement in ANCA-associated vasculitis: a multinational study

Rheumatology ◽  
2021 ◽  
Author(s):  
Sergey Moiseev ◽  
Andreas Kronbichler ◽  
Egor Makarov ◽  
Nikolay Bulanov ◽  
Matija Crnogorac ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Thromboembolic Events ◽  
The European Union ◽  
Similar Frequency ◽  
Anca Associated Vasculitis ◽  
Kidney Involvement ◽  
Venous Thromboembolic Events ◽  
Venous Thromboembolic

Abstract Objective To investigate the occurrence of venous thromboembolic events (VTE) in a large cohort of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) across the European Union, Turkey, Russia, UK and North America. Methods Patients with a definite diagnosis of AAV who were followed for at least 3 months and had sufficient documentation were included. Data on VTE, including either deep vein thrombosis or pulmonary embolism, were collected retrospectively from tertiary vasculitis centres. Univariate and multivariate regression models were used to estimate odds ratios (ORs) and 95% CIs. Results Over a median follow-up of 63 (interquartile range: 29, 101) months, VTE occurred in 278 (9.7%) of 2869 AAV patients with a similar frequency across different countries (from 6.3% to 13.7%), and AAV subtype [granulomatosis with polyangiitis: 9.8% (95% CI: 8.3, 11.6%); microscopic polyangiitis: 9.6% (95% CI: 7.9, 11.4%); and eosinophilic granulomatosis with polyangiitis: 9.8% (95% CI: 7.0, 13.3%)]. Most VTE (65.6%) were reported in the first-year post-diagnosis. Multiple factor logistic regression analysis adjusted for sex and age showed that skin (OR 1.71, 95% CI: 1.01, 2.92), pulmonary (OR 1.78, 95% CI: 1.04, 3.14) and kidney [eGFR 15–60 ml/min/1.73 m2, OR 2.86 (95% CI: 1.27, 6.47); eGFR &lt;15 ml/min/1.73 m2, OR 6.71 (95% CI: 2.94, 15.33)] involvement were independent variables associated with a higher occurrence of VTE. Conclusion Two-thirds of VTE occurred during the initial phase of active disease. We confirmed previous findings from smaller studies that a decrease in kidney function, skin involvement and pulmonary disease are independently associated with VTE.

Primary vasculitides

2018 ◽  
pp. 447-482
Author(s):  
Gavin Clunie ◽  
Nick Wilkinson ◽  
Elena Nikiphorou ◽  
Deepak R. Jadon
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Granulomatosis With Polyangiitis ◽  
Clinical Manifestations ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Consensus Conference ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Chapel Hill ◽  
Primary Angiitis ◽  
Chapel Hill Consensus Conference

This chapter describes large, medium, and small vessel primary vasculitides. The 2012 Chapel Hill Consensus Conference nomenclature of vasculitis is introduced, and acts as a framework for more detailed descriptions of polymyalgia rheumatic and giant cell arteritis, polyarteritis nodosa (systemic and cutaneous), granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, Takayasu arteritis, childhood-onset vasculitis, Kawasaki disease, Henoch–Schönlein purpura, and leucocytoclastic vasculitis. Other antineutrophil cytoplasmic antibody-associated vasculitides and primary angiitis of central nervous system in children are also covered. The epidemiology, aetiopathogenesis, classification criteria, presenting features, clinical manifestations, and management of these diseases are presented. In particular, diagnostic criteria for and mimics of polymyalgia rheumatic and giant cell arteritis are discussed in detail to permit rheumatologists and non-rheumatologists to confidently manage them.

Antineutrophil Cytoplasmic Antibody–Positive Vasculitis

Asthma ◽  
2014 ◽  
pp. 12-20
Author(s):  
Lanny J. Rosenwasser ◽  
Dennis K. Ledford
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Upper Airway ◽  
Airway Disease ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Anca Associated Vasculitis ◽  
Granulomatous Polyangiitis ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis

The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis syndromes— eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome or allergic angiitis with granulomatosis), granulomatous polyangiitis (Wegener’s granulomatosis), and microscopic polyangiitis—are serious comorbidities of asthma or upper respiratory disease, or the symptoms of the vasculitis may resemble asthma or associated upper airway disease. ANCA-associated vasculitis is potentially fatal but is responsive to a variety of treatments, if the vasculitis is recognized before serious organ dysfunction. The role of ANCA in these conditions has not been defined because eosinophilic granulomatosis with polyangiitis, granulomatous polyangiitis, or microscopic polyangiitis may occur without ANCA, and the titer of ANCA does not predict clinical course. Clinical suspicion and consideration of tissue biopsy are important for recognition before irreversible complications occur. Corticosteroid therapy for asthma or suspected asthma may modify the presentations of ANCA-positive vasculitis.

scholarly journals Review of guideline for the management of ANCA-associated vasculitis, presented in 2021 by the American College of Rheumatology/Vasculitis Foundation

2021 ◽  
Vol 59 (6) ◽  
pp. 684-692
Author(s):  
T. V. Beketova ◽  
I. Yu. Popov ◽  
V. V. Babak
Keyword(s):  
Autoimmune Diseases ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
American College Of Rheumatology ◽  
Anca Associated Vasculitis ◽  
Life Threatening ◽  
Eosinophilic Granulomatosis

The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are a group of severe life-threatening autoimmune diseases, and one of the most important problems in practical rheumatology. This article discusses 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of AAVs, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). The guideline features 26 recommendations and 5 upgraded position statements for GPA/ MPA, as well as 15 recommendations and 5 upgraded position statements for EGPA, which became the basis for the proposed algorithms for the treatment of patients with GPA/MPA and EGPA. Particular attention has been paid to the use of biologics.

scholarly journals Serum chitinase-3-like 1 protein is a useful biomarker to assess disease activity in ANCA-associated vasculitis: an observational study

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Sung Soo Ahn ◽  
Taejun Yoon ◽  
Yong-Beom Park ◽  
Maria Prendecki ◽  
Gurjeet Bhangal ◽  
...  
Keyword(s):  
Regression Analysis ◽  
Linear Regression ◽  
Disease Activity ◽  
Granulomatosis With Polyangiitis ◽  
Short Form ◽  
Linear Regression Analysis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Cytoplasmic Staining ◽  
Anca Associated Vasculitis ◽  
Sf 36

Abstract Background To investigate whether serum chitinase-3-like 1 protein (YKL-40) is associated with disease activity in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Methods ELISA was performed in serum samples from AAV patients who were enrolled in our prospective observational cohort to estimate levels of YKL-40. Birmingham vasculitis activity score (BVAS) (version 3), five factor score (FFS), and short form-36 (SF-36), as well as clinical and laboratory data were collected. Kidney expression of YKL-40 was assessed by immunohistochemical staining using renal biopsy tissues from ANCA-associated glomerulonephritis patients (AAGN). Severe AAV and FFS were defined as BVAS ≥ 12 and FFS ≥ 2, and the correlations between laboratory variables, BVAS, FFS, and SF-36 score were assessed using linear regression analysis. The optimal cut-off of serum YKL-40 for severe AAV and high FFS was calculated using the receiver operator characteristic curve analysis. Results Of the included 60 patients, 32 (53.3%), 17 (28.3%), and 11 (18.3%) were classified as microscopic polyangiitis, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis. The median BVAS and FFS were 7.0 and 1.0, whereas the mean SF-36 physical and mental component scores were 50.5 and 58.3. Serum YKL-40 level was higher in patients with severe AAV and high FFS compared to those without (p = 0.007 and p < 0.001); multivariable linear regression analysis revealed that serum YKL-40 was independently associated with BVAS, FFS, and SF-36 scores. On kidney tissues obtained from AAGN patients, strong cytoplasmic staining of YKL-40 was found in cells present in inflammatory lesions. In addition, AAV patients had higher levels of serum YKL-40 compared to those with systemic lupus erythematosus, rheumatoid arthritis, osteoarthritis, and healthy control. The proportion of patients having severe AAV and high FFS was significantly higher in those with serum YKL-40 > 221.3 ng/mL and > 227.1 ng/mL than those without (relative risk 2.852 and 7.000). In 12 patients with serial YKL-40 testing, 11 patients (91.7%) exhibited a reduction in serum YKL-40 levels following a decrease in disease activity (p < 0.001). Conclusion Our findings suggest that serum YKL-40 may be a clinically useful biomarker to assess AAV disease activity. Trial registration Retrospectively registered.

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