scholarly journals Chemokine/Cytokine Levels Correlate with Organ Involvement in PR3-ANCA-Associated Vasculitis

2021 ◽  
Vol 10 (12) ◽  
pp. 2715
Author(s):  
Janina Müller-Deile ◽  
Christian Jaremenko ◽  
Hermann Haller ◽  
Mario Schiffer ◽  
Marion Haubitz ◽  
...  
Keyword(s):  
Small Vessel Disease ◽  
Renal Involvement ◽  
Organ Involvement ◽  
Laboratory Marker ◽  
Renal Manifestation ◽  
Cytokines And Chemokines ◽  
Anca Associated Vasculitis ◽  
Kidney Involvement ◽  
Chemokine Ligand ◽  
Nose And Throat

Background: ANCA-associated vasculitis (AAV) is a rare small vessel disease characterized by multi-organ involvement. Biomarkers that can measure specific organ involvement are missing. Here, we ask whether certain circulating cytokines and chemokines correlate with renal involvement and if distinct cytokine/chemokine patterns can differentiate between renal, ear/nose/throat, joints, and lung involvement of AAV. Methods: Thirty-two sets of Birmingham vasculitis activity score (BVAS), PR3-ANCA titers, laboratory marker, and different cytokines were obtained from 17 different patients with AAV. BVAS, PR3-ANCA titers, laboratory marker, and cytokine concentrations were correlated to different organ involvements in active AAV. Results: Among patients with active PR3-AAV (BVAS > 0) and kidney involvement we found significant higher concentrations of chemokine ligand (CCL)-1, interleukin (IL)-6, IL21, IL23, IL-28A, IL33, monocyte chemoattractant protein 2 (MCP2), stem cell factor (SCF), thymic stromal lymphopoietin (TSLP), and thrombopoietin (TPO) compared to patients without PR3-ANCA-associated glomerulonephritis. Patients with ear, nose, and throat involvement expressed higher concentrations of MCP2 and of the (C-X-C motif) ligand-12 (CXCL-12) compared to patients with active AAV and no involvement of these organs. Conclusion: We identified distinct cytokine patterns for renal manifestation and for ear, nose and throat involvement of PR3-AAV. Distinct plasma cytokines might be used as non-invasive biomarkers of organ involvement in AAV.

scholarly journals AB0820 INFLUENCE OF ANCA ANTIBODIES ON DEMOGRAPHIC AND CLINICAL CHARACTERISTIC OF AAV

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1434.1-1434
Author(s):  
K. Wójcik ◽  
A. Masiak ◽  
Z. Zdrojewski ◽  
R. Jeleniewicz ◽  
M. Majdan ◽  
...  
Keyword(s):  
Renal Disease ◽  
End Stage Renal Disease ◽  
Clinical Characteristics ◽  
Renal Involvement ◽  
Organ Involvement ◽  
Unknown Etiology ◽  
Anca Associated Vasculitis ◽  
Stage Renal Disease ◽  
End Stage ◽  
Male To Female

Background:ANCA associated vasculitides (AAV) are a heterogeneous group of rare diseases with unknown etiology and the broad clinical spectrum ranging from life-threatening systemic disease, through single organ involvement to minor isolated skin changes. Unfortunately the clinical classification, ANCA specificity or genetic characteristics alone is not able to categorize AAV patients in a satisfactory manner. As a consequence advanced statistical techniques were used to identify and stratify AAV subphenotypes [1, 2]. Here we have analyzed influence of the ANCA type on clinical manifestations and demographic characteristics in various types of AAV, based on data from the POLVAS registryObjectives:We decided to retrospectively analyze a large cohort of Polish AAV patients deriving from several referral centers – members of the Scientific Consortium of the Polish Vasculitis Registry (POLVAS) – and concentrate on demographic and clinical characteristics of anti-PR3 and anti-MPO positive patients regardless of their clinical diagnosis.Methods:We conducted a systematic multicenter retrospective study of adult patients diagnosed with AAV between Jan 1990 and Dec 2016. Patients were enrolled by 9 referral centers. We analyzed dichotomous variables: gender; ANCA status – anti-PR3+ or anti-MPO+, ANCA negative; organ involvement - skin, eye, ENT, respiratory, heart, GI, renal, urinary, CNS, peripheral nerves and polytomous variable (number of relapses), supported by quantitative covariates (e.g., age at diagnosis, CRP at diagnosis, maximal serum creatinine concentration ever)[3].Results:MPO-positive patients (both GPA and EGPA phenotype) were older at the time of diagnosis with a substantial percentage diagnosed > 65 years of age, and with high rate of renal involvement. Interestingly, while in the whole group of patients diagnosed with EGPA male to female ratio was 1:2, the MPO+ EGPA patients showed M:F ratio of 1:1.The analysis of ANCA negative AAV reveled significant differences in GPA, ANCA negative group is characterized with significantly lower frequency of renal involvement compared to rest GPA (11,5% vs 63,7%) p<0,05 what should be emphasized ANCA negative AAV never lead to ESRD (end stage renal disease) or even transient dialysis.Conclusion:ANCA specificity is indispensable as a separate variable in any clinically relevant analysis of AAV subcategories. MPO+ group is characterized by older age at time of diagnosis, male to female ration 1:1, kidney involvement, and shows more homogenous clinical phenotype than PR3+ AAV patients. In our group ANCA negative AAV never lead to ESRD (end stage renal disease) or even transient dialysis.References:[1]Mahr A, Specks U, Jayne D. Subclassifying ANCA-associated vasculitis: a unifying view of disease spectrum. Rheumatol Oxf Engl 2019;58:1707–9. https://doi.org/10.1093/rheumatology/kez148.[2]Wójcik K, Biedroń G, Wawrzycka-Adamczyk K, Bazan-Socha S, Ćmiel A, Zdrojewski Z et al. Subphenotypes of ANCA-associated vasculitis identified by latent class analysis. Clin Exp Rheumatol. 2020 Sep 1. Epub PMID: 32896241.[3]Wójcik K, Wawrzycka-Adamczyk K, Włudarczyk A, Sznajd J, Zdrojewski Z, Masiak A, et al. Clinical characteristics of Polish patients with ANCA-associated vasculitides—retrospective analysis of POLVAS registry. Clinical Rheumatology. 1 wrzesień 2019;38(9):2553–63.Disclosure of Interests:None declared

Clinical Features and Outcomes of Immunoglobulin G4-related Disease

2021 ◽  
Author(s):  
Sua Lee ◽  
Chul Woo Yang
Keyword(s):  
Clinical Features ◽  
Clinical Laboratory ◽  
Renal Involvement ◽  
Multiple Organ ◽  
Organ Involvement ◽  
Response To Treatment ◽  
Clinical Feature ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Kidney Involvement

Abstract Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized disease, and a few epidemiologic studies about this disorder have been published. This research aimed to describe the clinical, laboratory, and histopathological features and outcomes of IgG4-RD. Ninety-four patients who satisfied the comprehensive diagnostic criteria on IgG4-RD were included in this study. Fifty-eight (61.7%) were men. The mean age was 54.8 years, and the median follow-up duration was 32.9 months. The clinical feature between single and multiple organ involvement and with or without kidney involvement groups were evaluated based on symptoms and laboratory findings. The clinical outcome was assessed according to treatment strategies and response. Of 94 patients, 56 (59.6%) had multiple organs involvement. Patients with multiple organ involvement had higher serum IgG and IgG4 levels than those with single organ involvement. Those with IgG4-related kidney disease (IgG4-RKD) had worse renal function, and the incidence of peripheral blood eosinophilia and hypocomplementemia was higher in patients with renal involvement than in those without. Glucocorticoids-based therapy was most commonly used. (79.8%). Thirty-nine (41.5%) achieved complete remission. Eighteen (19.1%) relapsed after response to treatment. IgG4-RD had different clinical features and outcomes. The number of organ involvement and kidney involvement were not associated with clinical outcomes.

scholarly journals POS0119 RENAL INVOLVEMENT AND NEED OF RENAL REPLACEMENT THERAPY IN ANCA ASSOCIATED VASCULITIS IN A SPANISH SINGLE-CENTER STUDY

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 270.2-271
Author(s):  
J. Álvarez Troncoso ◽  
J. C. Santacruz Mancheno ◽  
A. Díez Vidal ◽  
S. Afonso Ramos ◽  
A. Noblejas Mozo ◽  
...  
Keyword(s):  
Renal Replacement Therapy ◽  
Replacement Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Age At Diagnosis ◽  
Systemic Involvement ◽  
Risk Of Death ◽  
Anca Associated Vasculitis ◽  
The Mean

Background:Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EPGA). Renal involvement is frequent in AAV and is an important factor for morbidity and mortality.Objectives:The main objective of this study was to analyze the demographic, clinical, histological and therapeutic characteristics of renal involvement in patients with AAV and the risk of renal replacement therapy (RRT) or death.Methods:Retrospective observational study of 56 patients with AAV fulfilling classificatory criteria and renal involvement diagnosed between 1995 and 2020 from a Spanish tertiary centre. We studied the histological involvement (according to the 2010 classification in focal, crescentic, mixed or sclerotic), immunofluorescence (IF) and the treatment received with the risk of RRT or death.Results:We included 56 patients diagnosed with AAV and renal involvement. The mean age was 61.08±4.05 years; 58.9% were women. The mean follow-up time of these patients was 16.14± 8.80 years. Only 57.1% of patients presented systemic involvement.Most frequent non-renal AAV manifestations were lung involvement (39.3%), central nervous system (30.4%), otorhinolaryngology (ORL) (14.3%), skin (8.9%) and cardiac involvement (8.9%). Main immunological findings were ANCA-MPO+ (69.6%), ANCA-PR3+ (23.2%), ANCA-negative (5.4%). Low C3 was found in 19.6% patients. Histologic classification (HC) and need of RRT is described in table 1. Main HC in renal AAV was crescentic, mixed, focal and sclerotic respectively. Eight patients had not biopsy performed. IF was positive for C3 deposits in 20 patients (35.7%). Half of the patients presented <50% normal glomeruli.The treatment of renal involvement in AAV in our cohort was as follows: 83.9% (47) corticosteroids (CS) and cyclophosphamide (of which 40 received intravenous and 7 oral cyclophosphamide; and 12 patients associated plasma exchange (PE) with this treatment), 5.36% CS alone, 2 patients received CS and mycophenolate; 1 CS and rituximab, 1 CS and PE, and 2 patients received no treatment. A total of 13 patients received PE and 18 RRT. The mean time to RRT was 65.44±32.72 months. Relapses were not uncommon, 33.93% of the patients presented ≥1 relapse and 10.71% presented ≥2.Infections were very frequent since they were present in 91.07% of the patients. Other frequent non-immunological complications observed in the follow-up of these patients were thrombosis in 31.14%, cardiovascular events in 28.57% and cancer in 19.64%.Patients with ANCA-PR3+ were younger at diagnosis (p<0.001), were more likely to present cardiac (p=0.045) and ORL involvement (p<0.001). However, neither ANCA-PR3+ nor ANCA-MPO+ were specifically associated with the need of RRT or higher risk of death in our cohort. Use of CS alone for the treatment of renal AAV was associated with higher mortality (p=0.006) but CS and cyclophosphamide with lower mortality (p=0.044). ANCA-negative patients were more likely to receive no treatment. Having <50% normal glomeruli and C3 deposits on IF were associated with an increased need for RRT. Presenting focal disease on HC was protective for the need of RRT. Older age at diagnosis, systemic involvement of AAV and need of RRT was associated with higher mortality.Conclusion:AAVs are complex vasculitides with frequent renal involvement. Increased C3 deposition, non-focal histological forms, and <50% normal glomeruli were related to the need for RRT. In turn, the need for RRT, a later age at diagnosis, and systemic involvement were associated with higher mortality. Holistic and multidisciplinary early management of AAVs in experience centers can help improve renal prognosis and decrease mortality.References:[1]Binda et al. ANCA-associated vasculitis with renal involvement. J Nephrol. 2018 Apr;31(2):197-208.[2]Kronbichler et al. Clinical associations of renal involvement in ANCA-associated vasculitis. Autoimmun Rev. 2020 Apr;19(4):102495.Disclosure of Interests:None declared

scholarly journals FRI0223 SUBPHENOTYPES OF ANCA ASSOCIATED VASCULITIS IDENTIFIED BY LATENT CLASS ANALYSIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 694.3-695
Author(s):  
K. Wójcik ◽  
A. Ćmiel ◽  
A. Masiak ◽  
Z. Zdrojewski ◽  
R. Jeleniewicz ◽  
...  
Keyword(s):  
High Risk ◽  
Latent Class Analysis ◽  
Relapse Rate ◽  
Clinical Characteristics ◽  
Latent Class ◽  
Middle Age ◽  
Organ Involvement ◽  
Class Analysis ◽  
Fourth Class ◽  
Anca Associated Vasculitis

Background:ANCA associated vasculitides (AAV) are a heterogeneous group of rare diseases with unknown etiology and the clinical spectrum ranging from life-threatening systemic disease, through single organ involvement to minor isolated skin changes. Thus there is an unmet need for phenotype identification especially among patients with granulomatosis with polyangiitis GPA, patients with microscopic polyangiitis MPA group seems to be more uniform. Recently, based on previous clustering analysis and clinical, histopathological, serological and prognostic aspects three subcategories of AAV have been proposed and named as: non-severe AAV, severe PR3-AAV and severe MPO-AAV [1].Objectives:In line with these attempts to subcategorize AAV we decided to use latent class analysis (LCA) on a large multicenter cohort of polish AAV patients from POLVAS [2] registry to identify potential new subphenotypes or confirm already proposed ones.Methods:Latent Class Analysis (LCA) approach was used as a model based clustering method of objects described by dichotomous (e.g., gender; ANCA status – cANCA, pANCA; organ involvement - skin, eye, ENT, respiratory, heart, GI, renal, urinary, CNS, peripheral nerves) and polytomous (number of relapses) variables supported by quantitative covariates (e.g., age at diagnosis, CRP at diagnosis, maximal serum creatinine concentration ever).Results:Results of LCA on our AAV group returned four class model of AAV subphenotypes, confirming existence of the previously proposed by Mahr at al. [1] and revealed fourth – previously not described clinically relevant subphenotype. To this fourth class - belong patients only with GPA, diagnosed at young age, with multiorgan involvement, high relapse rate and relatively high risk of death.Table 1.AAV subcategorization – summary of clinical characteristics and ANCA specificityLCA Class 1LCA Class 2LCA Class 3LCA Class 4No of patients13019410297AAV typeMainly GPAMainly GPAmainly MPAOnly GPAAge at diagnosisMiddle ageMiddle ageOldYoungMale/female ratio1:22:11:11:1Main organ involvementENT, respiratory, eyeRenal, respiratory, ENTRenal, respiratory, skinMultiorgan involvementRelapse rateintermediateintermediatelowhighModified class description (based on ref. [1])Non severe AAVSevere PR3 AAVSevere MPO AAVSevere non-renalPR3 AAVConclusion:Based on multiple clinical and serological variables LCA methodology identified 4-class subphenotypes model of AAV. Fourth-class is a new clinically important subphenotype including exclusively PR3-positive young AAV patients with multiorgan involvement, high risk of relapse and distinct mortality.References:[1]Mahr A, Specks U, Jayne D. Subclassifying ANCA-associated vasculitis: a unifying view of disease spectrum. Rheumatol Oxf Engl 2019;58:1707–9.https://doi.org/10.1093/rheumatology/kez148.[2]Wójcik K, Wawrzycka-Adamczyk K, Włudarczyk A, Sznajd J, Zdrojewski Z, Masiak A, i in. Clinical characteristics of Polish patients with ANCA-associated vasculitides—retrospective analysis of POLVAS registry. Clinical Rheumatology. 1 wrzesień 2019;38(9):2553–63.Disclosure of Interests:Krzysztof Wójcik: None declared, Adam Ćmiel: None declared, Anna Masiak: None declared, Zbigniew Zdrojewski: None declared, Radoslaw Jeleniewicz: None declared, Maria Majdan Consultant of: Roche, Amgen, Speakers bureau: Roche, Amgen, Iwona Brzosko: None declared, Marek Brzosko: None declared, Piotr Głuszko: None declared, Małgorzata Stasiek: None declared, Małgorzata Wisłowska: None declared, Joanna Kur-Zalewska: None declared, Marta Madej: None declared, Anna Hawrot-Kawecka: None declared, Hanna Storoniak: None declared, Barbara Bułło-Piontecka: None declared, Alicja Dębska-Ślizień: None declared, Eugeniusz Kucharz: None declared, Katarzyna Jakuszko: None declared, Jacek Musiał: None declared

scholarly journals Henoch-Schönlein purpura outcome in children: A ten-year clinical study

2011 ◽  
Vol 139 (3-4) ◽  
pp. 174-178 ◽  
Author(s):  
Brankica Spasojevic-Dimitrijeva ◽  
Mirjana Kostic ◽  
Amira Peco-Antic ◽  
Divna Kruscic ◽  
Mirjana Cvetkovic ◽  
...  
Keyword(s):  
Renal Function ◽  
Kidney Biopsy ◽  
Renal Involvement ◽  
Immunosuppressive Treatment ◽  
Clinical Signs ◽  
Objective Evaluation ◽  
Severe Form ◽  
Renal Manifestation ◽  
Nephritic Syndrome ◽  
Nephrotic Range Proteinuria

Introduction. Henoch-Sch?nlein purpura (HSP) is the most common vasculitis of childhood. It is characterized by symptoms including nonthrombocytopenic purpura, abdominal pain, haematuria/proteinuria, and arthralgia/arthritis. The pleiomorphism of clinical signs in HSP could be confused with other conditions or other vasculitis forms. Objective. Evaluation of HSP clinical presentation, the onset and severity of renal manifestation in affected children and their outcome. Methods. A retrospective study of 49 patients diagnosed with HSP was conducted from September 1999 to September 2009. Children with severe renal manifestations (nephrotic range proteinuria, with or without nephrotic or nephritic syndrome) have undergone kidney biopsy. Results. Twenty-five patients developed renal manifestations after onset of the disease. In our study child?s older age was a risk factor for association with HSP nephritis. Six of the patients required kidney biopsy. They were successfully treated with various immunosuppressive protocols, as well as three of nine patients with nephrotic range proteinuria. Two patients developed most severe form of HSP nephritis, nephrotic-nephritic syndrome with histology grade IIIb/IVb. During the study period (average follow-up 6 years), all patients had a normal global renal function with mild proteinuria in only two cases. The prognosis of renal involvement was better than reports from other patient series. Conclusion. Long-term morbidity of HSP is predominantly attributed to renal involvement. During the study period, no patient had renal insufficiency or end stage renal disease after various combinations of immunosuppressive treatment. It is recommended that patients with HSP nephritis are followed for longer periods of time with a regular measurement of renal function and proteinuria.

PR3-ANCA-associated vasculitis is associated with a specific motif in the peptide-binding cleft of HLA-DP molecules

Rheumatology ◽  
2019 ◽  
Vol 58 (11) ◽  
pp. 1942-1949 ◽  
Author(s):  
Jon Waarst Gregersen ◽  
Christian Erikstrup ◽  
Per Ivarsen ◽  
Rie Glerup ◽  
Elizabeth Krarup ◽  
...  
Keyword(s):  
Amino Acid ◽  
Peptide Binding ◽  
Amino Acid Sequences ◽  
Homogeneous Population ◽  
Exon 2 ◽  
Hla Alleles ◽  
Hypervariable Regions ◽  
Anca Associated Vasculitis ◽  
Increased Risk ◽  
Kidney Involvement

Abstract Objectives This study aimed to characterize the association between HLA alleles and ANCA-associated vasculitis (AAV) in a genetically homogeneous population, and to analyse the contribution of specific HLA molecule amino acid sequences to the risk of AAV. Methods We included 187 Danish patients with AAV and 1070 healthy controls. All were HLA typed at two-field resolution. The association of HLA alleles to PR3- or MPO-AAV was analysed. The contribution of the dominant molecular motifs of the HLA-DPB1 molecule to the risk of AAV was investigated by association studies that included specific amino acid sequences of the hypervariable regions in exon 2. Results Ninety-four percent of patients with PR3-AAV were carriers of HLA-DPB1*04:01 while all patients with PR3-AAV were carriers of an HLA-DPB1*04 allele, and 85% were homozygous. This was significantly more than in the control group (P < 0.0001). The association was even stronger when HLA-DPB1*04:02 and -DPB1*23:01 were included. HLA-DPB1*04:01, -DPB1*04:02 and -DPB1*23:01 share amino acids in positions 8–9, 69, 76 and 84–87 within the hypervariable regions, but only positions 69 and 84–87 contributed significantly to the disease risk. HLA-DRB1*15 was associated with an increased risk of developing PR3-AAV, while HLA-DRB1*04, -DRB1*07 and -DQB1*03 were associated with a reduced risk of kidney involvement in PR3-AAV. MPO-AAV was only weakly associated with HLA class I alleles. Conclusion PR3-AAV is strongly associated with the HLA-DPB1 alleles HLA-DPB1*04:01, -DPB1*04:02 and -DPB1*23:01, which share amino acid sequences crucial for the peptide-binding groove.

scholarly journals MO038ANTIMYELOPEROXIDASE AND PROTEINASE 3 ANTIBODIES ARE A USEFUL MARKER AS NEPHRITIS FLARE PREDICTOR IN ANCA-ASSOCIATED VASCULITIS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Eva Rodriguez ◽  
Ana Belén Latze ◽  
Milagros Sierra ◽  
Ana María Romera ◽  
Diego Siedel ◽  
...  
Keyword(s):  
Roc Curve ◽  
Renal Involvement ◽  
Continuous Variable ◽  
Multiplex Assay ◽  
Anca Associated Vasculitis ◽  
Renal Flare ◽  
Necrotizing Glomerulonephritis ◽  
Renal Vasculitis ◽  
Renal Relapse ◽  
Antibody Levels

Abstract Background and Aims The diagnostic utility of ANCA antibodies in ANCA associated-vasculitis (AAV), antiproteinase 3 (PR3) and myeloperoxidase (MPO) testing is now undisputed, but the clinical utility of serial MPO/PR3 testing to predict relapses, remain controversial. The aim of this study was to analyze the relevance of serum MPO and PR3 antibody level assessment in the management of AAV. We sought to determine whether MPO and PR3 antibody levels correlated with renal disease activity, and whether an increase could predict a nephritis flare. Method Retrospective multicenter study including AAV-patients with renal involvement from 7 nephrology departments belonging to the Spanish Glomerular Study Group (GLOSEN). The main inclusion criteria were that MPO antibodies were detected by ELISA (Multiplex assay) and PR3 using chemioluminescence immunoassay. For statistical purpose a continuous variable were calculated, called delta MPO/PR3(ΔMPO y ΔPR3) that reflects change in antibodies levels 6 months before renal flare. Clinical data were recorded since complete remission of first nephritis diagnosis flare until second renal relapse. Results 113 AAV-patients with pauci-inmune necrotizing glomerulonephritis were included, 59 (52.2%) women, mean age (64.3±14.8 years), 85 patients (75.2%) MPO-AAV and 28 (24.8%) PR3-AAV, after a mean follow-up of 5±4.8 years, 54 renal relapses occurred in 40 (52,6%) MPO-AAV patients and in 14 (57.1%) PR3-AAVpatients. Serum MPO levels were significant higher in relapser-patients compared with non- relapser patients, 3±1.2 months before nephritis relapse [(n=32) 19.2±12.2 IA vs (n=38) 3.2±5.1 IA, p&lt;0.001)]. Δ MPO levels were significant higher in relapse-patients compared with non-relapser patients [(n=32) 8.3±12 IA vs (n=38) 0.9±3.1.1 IA, p=0.001). The discrimination value of the MPO antibody levels 3 months before renal relapse were established by means of a ROC curve: AUC of 0.82 (95% CI 0.73 to 0.92; p&lt;0.001) and the MPO cut-off value predicting renal relapse were established in 8.3 IA. The discrimination value of the ΔMPO were established by means of ROC curve: AUC of 0.76 (95% CI 0.63 to 0.88; p&lt;0.001), ΔMPO cut-off value were established in an increase of MPO levels of 3.7 IA. Serum PR3 levels were significant higher in relapser-patients 2.8±1.4 months before relapse [(n=14) 58.6±6.6 IA vs (n=7) 2.0±0.6 IA, p&lt;0.001)] and Δ PR3 levels were significant higher in relapse-patients compared with non-relapser patients [(n=14) 57.5±28.5 IA vs (n=7) 0.8±0.2. IA, p&lt;0.001)]. The discrimination value of the PR3 3 months before flare and Δ PR3 antibody levels were established by means of ROC curve: AUC 1. Conclusion Our results show that MPO antibodies, measured by Multiplex assay, could be a useful predictor of glomerulonephritis flare in AAV-patients. MPO levels 3 months before renal flare seems a good marker confirmed by ROC curve results, MPO cut-off value with the best sensitivity and specificity in predicting renal relapse was established in 8.3 IA. Δ MPO levels show that increase in titers ≥ 3.7 IA 6 months before renal flare, predict nephritis relapse However, PR3 antibodies are not a useful predictor marker, rise in antibodies level indicate renal vasculitis activity.

scholarly journals All-cause and cause-specific mortality in ANCA-associated vasculitis: overall and according to ANCA type

Rheumatology ◽  
2019 ◽  
Vol 59 (9) ◽  
pp. 2308-2315 ◽  
Author(s):  
Zachary S Wallace ◽  
Xiaoqing Fu ◽  
Tyler Harkness ◽  
John H Stone ◽  
Yuqing  Zhang ◽  
...  
Keyword(s):  
Causes Of Death ◽  
Cox Regression ◽  
Renal Involvement ◽  
Inception Cohort ◽  
Vital Status ◽  
Anca Associated Vasculitis ◽  
Common Causes ◽  
Mortality Incidence ◽  
Standardized Mortality Ratios

Abstract Objective The objective of this study was to evaluate causes of death in a contemporary inception cohort of ANCA-associated vasculitis patients, stratifying the analysis according to ANCA type. Methods We identified a consecutive inception cohort of patients newly diagnosed with ANCA-associated vasculitis from 2002 to 2017 in the Partners HealthCare System and determined vital status through the National Death Index. We determined cumulative mortality incidence and standardized mortality ratios (SMRs) compared with the general population. We compared MPO- and PR3-ANCA+ cases using Cox regression models. Results The cohort included 484 patients with a mean diagnosis age of 58 years; 40% were male, 65% were MPO-ANCA+, and 65% had renal involvement. During 3385 person-years (PY) of follow-up, 130 patients died, yielding a mortality rate of 38.4/1000 PY and a SMR of 2.3 (95% CI: 1.9, 2.8). The most common causes of death were cardiovascular disease (CVD; cumulative incidence 7.1%), malignancy (5.9%) and infection (4.1%). The SMR for infection was greatest for both MPO- and PR3-ANCA+ patients (16.4 and 6.5). MPO-ANCA+ patients had an elevated SMR for CVD (3.0), respiratory disease (2.4) and renal disease (4.5). PR3- and MPO-ANCA+ patients had an elevated SMR for malignancy (3.7 and 2.7). Compared with PR3-ANCA+ patients, MPO-ANCA+ patients had a higher risk of CVD death [hazard ratio 5.0 (95% CI: 1.2, 21.2]; P = 0.03]. Conclusion Premature ANCA-associated vasculitis mortality is explained by CVD, infection, malignancy, and renal death. CVD is the most common cause of death, but the largest excess mortality risk in PR3- and MPO-ANCA+ patients is associated with infection. MPO-ANCA+ patients are at higher risk of CVD death than PR3-ANCA+ patients.

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