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 ◽  
...  

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

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 345.1-346
Author(s):  
A. Berti ◽  
G. Boleto ◽  
P. A. Merkel ◽  
G. Tomasson ◽  
S. Monti ◽  
...  

Background:The OMERACT Vasculitis Working Group has defined a Core Domain Set of outcome measures for ANCA-associated vasculitis (AAV). However, the psychometric properties of available outcome measurement instruments in AAV, an essential consideration when choosing among instruments, have not been summarized.Objectives:To systematically review and summarize the psychometric properties of outcome measurement instruments used in AAV.Methods:A comprehensive search of several databases (Medline, EMBASE, Cochrane, Scopus, among others) from inception to July 14, 2020 and without language limitations was conducted. Articles were included if they covered psychometric properties of instruments used in AAV (granulomatosis with polyangiitis, GPA; microscopic polyangiitis, MPA; eosinophilic granulomatosis with polyangiitis, EGPA); articles encompassing other systemic vasculitides and not presenting the data for AAV separately were excluded. Following the COSMIN and OMERACT frameworks, different psychometric properties (validity, inter- and intra-observer reliability, sensitivity to change, and feasibility) of outcome measurement instruments used in AAV were assessed. Risk of bias was assessed according to the COSMIN checklist.Results:From 2505 articles identified, 20 met the predefined criteria. Three were identified as development studies, 14 were validation studies, and 3 pursued both objectives.These studies provided information on 16 instruments: 8 assessing disease activity, 1 assessing disease damage, 3 assessing patient-reported outcome, 4 assessing function (Figure 1). Overall, a few psychometric properties have been considered in each study, ranging from one to five. Most of the instruments were tested in GPA only (n=7), followed by AAV as a group (GPA, MPA and EGPA; n=5), MPA and GPA (n=3), and EGPA only (n=1). Sample sizes of the studies ranged between 27 and 626 patients. The studies with a higher risk of bias, according to COSMIN definitions, were those assessing RAPID3, MVIA, ENT/GPA DAS, and ODSS.There was a wide heterogeneity of the psychometric proprieties assessed for each instrument. Validity was the most frequently assessed domain in 88% of the instruments, and few properties other than construct validity were reported (Figure 1).Within each domain, BVAS/WG for activity, VDI for damage, AAV-PRO for patient-reported outcomes, and ODSS for function were the instruments with more psychometric features assessed. For the disease activity domain, BVAS/WG showed a good validity having the highest correlation with physician global assessment (r=0.90), a good reliability (intra-observer ICC=0.62; inter-observer ICC=0.97), and good feasibility and responsiveness. For disease damage, VDI showed moderate validity (correlations with BVAS/WG at 5-year with r=0.20 and BVAS/WG at 1-year with r=0.40) and good feasibility. Among patient-reported outcomes, AAV-PRO had the best performance in terms of validity (construct validity: correlations of the 6 disease domains and EQ-5D-5L, with r ranging between -0.78 and -0.55; discriminating validity between active disease versus remission of the 6 disease domains, p<0.0001 for all comparisons). The performance of instruments assessing function domain was low-to-moderate.Conclusion:Sixteen instruments covering the OMERACT domains of disease activity, damage, patient-reported outcome, and function had their psychometric properties assessed in the study of AAV. The majority were developed or validated for GPA only or AAV as a group. Overall, validity was the domain most frequently assessed. BVAS/WG, VDI, AAV-PRO, and ODSS were the instruments with more psychometric features assessed. More rigorous studies aimed at estimating a wider range of psychometric properties in larger numbers of patients with AAV are warranted.References:[1]Castrejon I, et al. Clin Exp Rheumatol. 2015[2]Merkel PA, Journal of Rheumatology, July 2011Disclosure of Interests:None declared


2021 ◽  
Author(s):  
Irena Doubelt ◽  
Jason M. Springer ◽  
Tanaz A. Kermani ◽  
Antoine G. Sreih ◽  
Cristina Burroughs ◽  
...  

BACKGROUND Patient-based registries can help advance research in rare diseases such as eosinophilic granulomatosis with polyangiitis (EGPA), a complex, multi-organ form of anti-cytoplasm neutrophil antibody (ANCA)-associated vasculitis. OBJECTIVE To compare patient-reported vs. physician-reported manifestations, treatments, and outcomes for patients with EGPA. METHODS Comparative analysis of patients ≥18 years with EGPA in Canada or the United States from two separate cohorts: i) The Vasculitis Patient-Powered Research Network (VPPRN), a self-enrolled, secure portal with patient-entered data updated quarterly (2013-2019), vs. ii) The Vasculitis Clinical Research Consortium (VCRC) observational studies, a physician-entered database (2003-2019) of patients who fulfilled the 1990 American College of Rheumatology classification criteria for EGPA. Studied parameters included demographics, clinical manifestations, ANCA status, treatments, and relapses. RESULTS Data from 195 patients with a validated diagnosis of EGPA in the VPPRN and 354 patients enrolled in the VCRC were analyzed. Compared to the VCRC cohort, the patients in the VPPRN cohort were more commonly female (69.2% vs. 59.0% in the VCRC cohort; P =.02), younger at diagnosis (47.3 vs. 50.0 years; P =.03), reported similar frequencies of asthma (96.2% vs 92.9% in VCRC; P =.13), cardiac manifestations (28.8% vs 21.2%; P =.06), but less frequent lung manifestations other than asthma, and more frequent disease manifestations in all other organ systems. ANCA positivity was 48.9% in the VPPRN patients vs. 38.9% (P=.05) in the VCRC cohort. Relapsing disease after study enrollment was reported in 32.3% patients in the VPPRN compared 35.7% of patients in the VCRC. Most therapies (glucocorticoids, cyclophosphamide, mepolizumab) were used at similar frequencies in both groups, except for rituximab with VPPRN patients reporting more use than VCRC cohort (24.1% vs. 10.5%; P =<.001). CONCLUSIONS Patients with EGPA generally report having more manifestations of disease than physicians report for patients with EGPA. These differences imply the need to reconsider how patient- and physician-reported data are collected for the study of EGPA, and reevaluate disease specific definitions. CLINICALTRIAL ClinicalTrials.gov: (1) VCRC Longitudinal Study (LS) NCT00315380 https://clinicaltrials.gov/ct2/show/NCT00315380 and (2) One-Time DNA (OT) study NCT01241305 https://clinicaltrials.gov/ct2/show/NCT01241305


2015 ◽  
Vol 75 (4) ◽  
pp. 715-720 ◽  
Author(s):  
Polina Putrik ◽  
Sofia Ramiro ◽  
Monika Hifinger ◽  
Andras P Keszei ◽  
Ihsane Hmamouchi ◽  
...  

ObjectivesTo investigate patterns in patient-reported and physician-reported disease outcomes in patients with rheumatoid arthritis (RA) from countries with different level of socioeconomic development.MethodsData from a cross-sectional multinational study (COMOrbidities in RA) were used. Contribution of socioeconomic welfare (gross domestic product (GDP); low vs high) of country of residence to physician-reported (tender joint count, swollen joint count (SJC), erythrocyte sedimentation rate, disease activity score based on 28 joints assessment (DAS28)-3v based on these three components and physician global assessment) and patient-reported (modified Health Assessment Questionnaire (mHAQ), patient global assessment and fatigue) disease outcomes was explored in linear regressions, adjusting for relevant confounders.ResultsIn total, 3920 patients with RA from 17 countries (30 to 411 patients per country) were included, with mean age of 56 years (SD13) and 82% women. Mean SJC varied between 6.7 (Morocco) and 0.9 (The Netherlands), mean mHAQ ranged between 0.7 (Taiwan) and 1.5 (The Netherlands). Venezuela had the lowest (1.7) and the Netherlands the highest score on fatigue (5.0). In fully adjusted models, lower GDP was associated with worse physician-reported outcomes (1.85 and 2.84 more swollen and tender joints, respectively, and 1.0 point higher DAS28-3v), but only slightly worse performance-based patient-reported outcome (0.15 higher mHAQ), and with better evaluation-based patient-reported outcomes (0.43 and 0.97 points lower on patient global assessment and fatigue, respectively).ConclusionsIn patients with RA, important differences in physician-reported and patient-reported outcomes across countries were seen, with overall a paradox of worse physician-reported outcomes but better patient-reported outcomes in low-income countries, while results indicate that these outcomes in multinational studies should be interpreted with caution. Research on explanatory factors of this paradox should include non-disease driven cultural factors influencing health.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1548-1548
Author(s):  
Y. Liu ◽  
L. MA ◽  
L. Jiang

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


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 92.2-93
Author(s):  
J. Tieu ◽  
S. Lester ◽  
W. Raymond ◽  
H. Keen ◽  
C. Hill ◽  
...  

Background:The increased risk of malignancy in patients with ANCA-associated vasculitis (AAV) and polyarteritis nodosa (PAN) has been attributed to late treatment related effects, with non-melanoma skin and genitourinary cancers most frequently reported in European studies1,2,3. Malignancy has not been examined in patients with AAV/PAN in Australia, where environmental factors may influence risk.Objectives:To determine the risk and timing of incident cancer in Western Australian (WA) AAV/PAN patients compared to controls.Methods:Patients and controls were ascertained through the WA Hospital Morbidity Data collection System (HMDS). Administrative hospitalisation data were linked with the WA cancer and death registries. Data was available between 1980-2015. Patients were classified into two sub-groups using International Classification of Disease (ICD) -9 and/or -10 codes: (1) GPA/MPA- granulomatosis with polyangiitis (GPA)/microscopic polyangiitis (MPA), and (2) other-AAV/PAN- eosinophilic granulomatosis with polyangiitis (EGPA), PAN, and other patients with any AAV or PAN where more specific ICD-10 coding was not available. Controls were age, sex and temporally matched (at patient diagnosis date) and had no rheumatological diagnosis. Patients and controls with prior cancer were excluded from the analysis.Spline-based estimation of time-varying hazard ratios (HR) for incident cancer in patientsvscontrols was performed using the Stata library stpm2cr4. Results for cause-specific models, which treated deaths in patients without cancer as censored, were confirmed using models treating death as a competing risk. The risk of specific cancers was analysed by Cox regression.Results:The analysis included 391 patients (165 GPA/MPA, 217 other-AAV/PAN) and 4913 controls, with 86 incident cancers (over 3556.7 person-years) observed in patients and 1119 (over 64997.0 person-years) in controls. Patients and controls were well matched for age (mean ± standard deviation GPA/MPA: 55 ± 18 years, other AAV/PAN: 59 ± 17 years, controls 57 ± 16 years), and sex (female: GPA/MPA 48%, other AAV/PAN 46%, controls 46%).Incident cancer risk and timing differed between the two patient subgroups (Figure 1). The risk of incident cancer in GPA/MPA patients, compared to controls, increased with disease duration, whilst other-AAV/PAN patients had a greater risk within the first two years of diagnosis, but a similar risk to controls in the longer term.By specific cancers, GPA/MPA patients had an increased risk of skin cancers (excluding squamous and basal cell carcinomas): hazard radio (HR) 2.71 95% confidence interval (CI) 1.55 – 4.74, and genitourinary cancers: HR 3.64, 95% CI 1.58, 8.39, which was not observed in other-AAV/PAN patients. While there was trend for an overall increase in haematological cancers, this was inconclusive.Conclusion:Incident cancer risk, driven by skin and genitourinary cancers, increased with disease duration in GPA/MPA patients, consistent with previous studies, suggestive of a treatment related effect. In contrast, cancer was more frequently observed early after diagnosis in other-AAV/PAN patients. Our findings suggest that vigilance for incident cancers is required for all patients with AAV and PAN after diagnosis and in long term management, considering distinct periods of greater risk by disease subgroup.References:[1]Heijl C et al. Ann Rheum Dis 2011;70:1415-1421[2]Lafarge A et al. Ann Rheum Dis 2019;0:1-2[3]Farschou M et al. Rheumatology 2015;54:1345-1350[4]Mozumder S et al. Stata J. 2017;17(2):462-489Disclosure of Interests:Joanna Tieu: None declared, Susan Lester: None declared, Warren Raymond: None declared, Helen Keen Speakers bureau: Pfizer Austrlaia, Abbvie Australia, Catherine Hill: None declared, Johannes (“Hans”) Nossent Speakers bureau: Janssen


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1671.1-1671
Author(s):  
L. Brites ◽  
L. Saraiva ◽  
F. Costa ◽  
J. Dinis de Freitas ◽  
M. Luis ◽  
...  

Background:Patient global Assessment (PGA) of disease activity is considered a key patient reported outcome in Rheumatoid Arthritis (RA) and Psoriatic Arthritis (PsA), both being included in combined indices of disease activity. However, patients and physicians frequently disagree in their assessment.Objectives:This study aimed at comparing the degree of this discrepancy and its determinants in RA and PsA.Methods:Cross sectional study including 100 patients with RA (ACR/EULAR 2010 criteria) and 100 patients with PsA with predominant peripheral joint involvement (CASPAR criteria), aged ≥18 years, randomly selected from the electronic registry Reuma.pt. Data were collected from the most recent rheumatology visit during the last year: sociodemographic data, disease duration (years), tender and swollen joint counts 0-28 (TJC and SJC), disease activity (DAS28 3V-PCR), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), patient’s pain assessment, PGA and physician global assessment (PhGA). The discrepancy between patients and physicians (ΔPPhGA) was defined as PGA minus PhGA, and a difference > |20mm| was taken as “discordance”. Categorical variables are presented as proportions and continuous variables as mean (±SD). Patient and clinical characteristics were compared between patients with RA and PsA using t- test and χ2 test, as adequate. Variables with p<0.05 or clinically relevant were included in multivariable logistic regression analysis to identify correlates for ΔPPhGA in the whole sample. A p≤0.05 was considered statistically significant.Results:Compared to PsA, patients with RA were more often female (90% Vs 49%,p< 0.05), older (66.7 ± 10.7 Vs 58.3 ± 12.2 years,p< 0.05) and had a shorter disease duration (18.2 ± 9.8 Vs 19.9 ± 9.7 years,p= 0.202). Regarding disease activity, the RA and PsA groups were comparable: DAS28 3V-PCR (2.3 ± 0.9 Vs 2.4 ± 1.0,p= 0.34). Patients with RA had a higher mean ΔPPhGA (30.4 ± 30.6 Vs 25.4 ± 27.5,p< 0.05), and were more frequently discordant to the physician (69% Vs 51%,p< 0.05). In univariable analysis, having RA, higher patient’s pain assessment and higher ESR were associated to patient-physician discordance. In multivariable analysis, only patient’s pain assessment (OR 1.04 [95% CI 1.03-1.06], p = 0.00) and TJC (OR 0.82 [95% CI 0.68-0.97], p = 0.02) remained as predictors of discordance.Conclusion:Despite comparable disease activity scores in RA and PsA patients, RA patients tend to have a worst self-perception of their disease activity compared to their physician´s. Patient’s pain assessment and TJC were the only predictors of patient-physician discordance, irrespective of the disease.Disclosure of Interests:Luisa Brites: None declared, LILIANA SARAIVA: None declared, Flavio Costa: None declared, João Dinis de Freitas: None declared, Mariana Luis: None declared, Ana Rita Prata: None declared, Helena Assunção: None declared, José Antonio P. da Silva Grant/research support from: Pfizer, Abbvie, Consultant of: Pfizer, AbbVie, Roche, Lilly, Novartis, João Rovisco: None declared, Catia Duarte: None declared


Lupus ◽  
2021 ◽  
pp. 096120332110279
Author(s):  
Worawit Louthrenoo ◽  
Nuntana Kasitanon ◽  
Eric Morand ◽  
Rangi Kandane-Rathnayake

Objective To determine longitudinal associations between Physician Global Assessment (PGA) and patient-reported outcomes (PROs) in patients with systemic lupus erythematosus (SLE). Methods Patients attending a rheumatology clinic between 2013 and 2017 completed specific (SLEQOL) and generic (SF36) health-related quality of life (HRQoL) surveys and rated their global rating of change (GRC) at each visit. PGA, SLEDAI-2K and SLE Flare Index (SFI) were also captured on all visits. Generalised estimating equations (GEE) methods were used to examine longitudinal associations of PGA with PROs and clinical indicators. Results 337 patients were followed for a median [IQR] of 3.2 [1.6, 3.4] years (2,059 visits). High PGA (>1) was strongly associated with high SLEDAI-2K scores, the presence of flares and poor PROs. Odd ratios (OR) [95% CI] of PGA > 1 in patients with SLEDAI-2K >4 & <10 and SLEDAI-2K ≥10, compared to SLEDAI-2K ≤ 4, were 3.46 [2.36, 5.08], p < 0.001 and 6.39 [4.30, 9.49], p < 0.001, respectively. OR [95% CI] of PGA > 1 in patients with mild-to-moderate or severe flares were 2.09 [1.62, 2.71], p < 0.001 and 4.42 [3.21, 6.07], p < 0.001, respectively. Mental components of both SLEQOL (mood, self-image) and SF36 (MCS) surveys demonstrated significant associations with high PGA. After adjusting for SLEDAI-2K, one-point increase in PGA was associated with reductions in SLEQOL total score and SF36-MCS by 2.33 (regression coefficient (RC) [95% CI] = −2.33 [−3.77, −0.88], p = 0.002), and 4.16 (RC [95% CI] = −4.16 [−5.19, −3.13], p < 0.001) points, respectively. Associations of some physical components (SLEQOL-symptoms, and SF36-PCS) with PGA attenuated when adjusted for SLEDAI-2K. Patients who rated low scores of GRC, which indicate health deterioration, were twice as likely to have PGA > 1 (OR [95%CI] 1.99 [1.25, 3.16], p = 0.004). Conclusion High PGA was strongly associated with poor mental health, high disease activity and flares. This study confirms the value of PGA as an efficient assessment tool for SLE.


2020 ◽  
Author(s):  
Yun Liu ◽  
Lili Ma ◽  
Zongfei Ji ◽  
Rongyi Chen ◽  
Xiufang Kong ◽  
...  

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.


2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Sung Soo Ahn ◽  
Taejun Yoon ◽  
Yong-Beom Park ◽  
Maria Prendecki ◽  
Gurjeet Bhangal ◽  
...  

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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