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OBJECTIVES: The study aimed to characterise the Polish population of (ANCA)-associated vasculitides (AAV) with respiratory involvement (RI), in comparison to the subgroup without lung manifestations and the other cohorts. METHODS: Retrospective analysis of the Polish population of AAV with RI was conducted, based on data from the POLVAS registry. Standard descriptive statistics, χ2 test, and Mann-Whitney U test were used to perform comparisons. RESULTS: Among 461 cases qualified to this study, there were 316 cases with RI (68.5%), 206 with granulomatosis with polyangiitis (GPA) (65.2%), 80 with eosinophilic granulomatosis with polyangiitis (EGPA) (25.3%) and 30 with microscopic polyangiitis (MPA) (9.5%). Proportion of RI in GPA, MPA, and EGPA accounted for 67.8%; 40.0%; 97.6%, respectively. The number of relapses was higher in the RI group (median 1.0 vs. 0.0; p=0.01). In the subgroup of combined GPA and MPA with RI, the trends toward higher proportion of deaths (11.7% vs. 5.7%; p=0.07), relapses requiring hospitalisation (52.2% vs. 42.4%, p=0.07) and relapses requiring admission to the intensive care unit (5.6% vs. 1.4%, p=0.09) were observed, median maximal concentration of CRP was higher (46 vs. 25 mg/l; p=0.01) and more aggressive treatment was administered. CONCLUSIONS: Prevalence of RI in the Polish population of AAV is similar to the values reported in the literature, however, the proportion observed in GPA is closer to those presented in Asian than Western European cohorts. RI seems to be associated with a more severe course of disease and its presence prompts more aggressive treatment.

Wstęp:Rozpoczęcie leczenia nerkozastępczego wiąże się zasadniczo ze zmianą stylu odżywiania. Istotnym problemem klinicznym, często obserwowanym u chorych hemodializowanych, jest dodatni bilans fosforanowy. Postępowanie w przypadku hiperfosfatemii opiera się przede wszystkim na interwencji dietetycznej i stosowaniu leków wiążących fosfor w przewodzie pokarmowym. Właściwie przeprowadzona edukacja chorych na PCHN ułatwia postrzeganie zagrożeń, jakie wynikają z niewłaściwej diety.Materiał i metody:W badaniu zastosowano metodę sondażu diagnostycznego z wykorzystaniem kwestionariusza ankiety własnego autorstwa. Sondaż zawierał pytania zamknięte dotyczące znajomości wskazówek dietetycznych zalecanych chorym hemodializowanym, pozwalających na utrzymanie zalecanej fosfatemii. Analizę statystyczną wykonano w programie SAS wersja 9.4 (SAS Institute Inc., Cary, North Carolina, USA), przyjmując poziom istotności statystycznej p < 0,05. Wykorzystano testy: Chi2/Fishera z uwagi na małą liczebność w podgrupach, test Kruskala-Wallisa dla porównania trzech grup oraz test U Manna-Whitneya dla porównania dwóch grup.Wyniki:Chorzy hemodializowani byli świadomi konieczności stosowania ograniczeń w spożywaniu produktów z wysoką zawartością fosforu, lecz mieli problemy z wybraniem tych, które są zalecane przy komponowaniu posiłków. Właściwy zestaw mięs wybrało 44 chorych (36%). Spożycie nabiału w umiarkowanej ilości deklarowały 74 osoby (61%). Spożycie żółtka jaja kurzego ograniczyło 46 respondentów (38%). Z puli dostępnych serów spożycie głównie twarogu deklarowało 92 badanych (76%). Właściwy zestaw pieczywa wskazało 31 pacjentów (26%), a napojów gazowanych unikało 73 chorych (60%).Wnioski:Stan wiedzy chorych na temat możliwości zapobiegania hiperfosfatemii był dalece niewystarczający.

INTRODUCTION ANCA-associated vasculitides (AAV) is a group of rare disorders where inflammation and damage of the small blood vessels lead to dysfunction of the supplied organs. In severe flares of the disease patients may require intensive care unit (ICU) admission and treatment. The study aims to characterize Polish patients with AAV who were admitted to the ICU and compare them to the others. MATERIAL AND METHODS An observational, retrospective study based on the POLVAS - registry of Polish adult patients with AAV was carried out. Patients admitted to the ICU (ICU group) were identified and compared with the patients who did not require ICU admission (non-ICU group). Characteristics and comparison between groups were made using standard statistic descriptive methods. RESULTS 30 patients admitted to the ICU were identified among 573 cases included in the registry. All patients in the ICU group with available data were ANCA positive. The clinical manifestations related to the ICU admission were respiratory, renal and central nervous system involvement. The treatment regimen for remission induction was similar in both groups. Almost half of the patients in the ICU-group (48.3%) required dialysis, whereas in the non-ICU group it was 21.8% (P = 0.01). Infections were also more frequent in the ICU group (72.4% vs. 36.9% P < 0.001). The mortality rate among patients who needed ICU treatment was significantly higher when compared to the rest of the patients (53.6% vs. 7.8%; P < 0.001). CONCLUSIONS In the Polish AAV cohort one in twenty patients required ICU admission. This group was characterized by multiple organ involvement and high mortality.

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%) pConclusion: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

Purpose The aim of this study is to present the treatment modalities and associated side effects in a Polish nation-wide ANCA-associated vasculitides (AAV) patients’ cohort. Materials and methods Retrospective analysis of patients diagnosed with AAV between 1990 and 2016, included in the POLVAS registry was performed. Standard descriptive statistic methods were used with an emphasis on the treatment modalities. Results There were 625 patients diagnosed with AAV included in this study: 417 cases of granulomatosis with polyangiitis (GPA; 66.7%), 106 cases of microscopic polyangiitis (MPA; 17.0%) and 102 cases of eosinophilic granulomatosis with polyangiitis (EGPA; 16.3%). The mean age at the date of diagnosis was 50.4 (±15.7) years and the median observational period amounted to 4.0 (2.0–8.0) years. Glucocorticosteroids (GCs) were the medicaments most frequently used for remission induction (593/622; 95.3%), followed by cyclophosphamide (487/622; 78.3%), rituximab (44/622; 7.1%), and methotrexate (39/622; 6.3%). GCs were also most frequently administered for maintenance therapy (499/592; 84.3%), followed by azathioprine (224/592; 37.8%), methotrexate (136/592; 23.0%) and mycophenolate mofetil (99/592; 16.7%). The median cumulative doses of cyclophosphamide and rituximab equalled 7.99 g (4.18–14.0) and 2000 mg (1500–2800), respectively. The most commonly observed adverse events included: infections - 214/551 cases (38.8%), which were associated with the time of observation (OR = 1.05; 95% CI 1.01–1.10), the use of GCs intravenous pulses (OR = 2.76; 95% CI 1.68–4.54) and need for haemodialysis (OR = 1.73; 95% CI 1.10–2.71). Conclusions Polish patients with AAV were predominantly treated according to appropriate guidelines. The most frequent adverse events were typical for usually administered immunosuppressive treatment.

Objective Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare small to medium-size vessel systemic diseases. As their clinical picture, organ involvement, and factors influencing outcome may differ between countries and geographical areas, we decided to describe a large cohort of Polish AAV patients coming from several referral centers-members of the Scientific Consortium of the Polish Vasculitis Registry (POLVAS). Methods We conducted a systematic multicenter retrospective study of adult patients diagnosed with AAV between Jan 1990 and Dec 2016 to analyze their clinical picture, organ involvement, and factors influencing outcome. Patients were enrolled to the study by nine centers (14 clinical wards) from seven Voivodeships populated by 22.3 mln inhabitants (58.2% of the Polish population). Results Participating centers included 625 AAV patients into the registry. Their distribution was as follows: 417 patients (66.7%) with GPA, 106 (17.0%) with MPA, and 102 (16.3%) with EGPA. Male-to-female ratios were almost 1:1 for GPA (210/207) and MPA (54/52), but EGPA was twice more frequent among women (34/68). Clinical manifestations and organ involvement were analyzed by clinical phenotype. Their clinical manifestations seem very similar to other European countries, but interestingly, men with GPA appeared to follow a more severe course than the women. Fifty five patients died. In GPA, two variables were significantly associated with death: permanent renal replacement therapy (PRRT) and respiratory involvement (univariate analysis). In multivariate analysis, PRRT (OR = 5.3; 95% confidence interval (CI) = 2.3–12.2), respiratory involvement (OR = 3.2; 95% CI = 1.06–9.7), and, in addition, age > 65 (OR = 2.6; 95% CI = 1.05–6.6) were independently associated with death. In MPA, also three variables were observed to be independent predictors of death: PRRT (OR = 5.7; 95% CI = 1.3–25.5), skin involvement (OR = 4.4; 95% CI = 1.02– 19.6), and age > 65 (OR = 6.3; 95% CI = 1.18–33.7). Conclusions In this first multicenter retrospective study of the Polish AAV patients, we have shown that their demographic characteristics, disease manifestations, and predictors of fatal outcome follow the same pattern as those from other European countries, with men possibly suffering from more severe course of the disease.

WstępWydaje się, że zespół metaboliczny (MS) jest dodatkowym czynnikiem zwiększającym ryzyko złamań osteo-porotycznych. Celem tego badania była ocena wpływu zespołu metabolicznego na metabolizm kości i ryzyko złamań osteoporotycznych u otyłych mężczyzn i kobiet.Materiał i metodyW badaniu wzięło udział 40 otyłych mężczyzn i 40 otyłych kobiet. Badanych podzielono na 2 grupy: pacjentów z otyłością bez MS (20 mężczyzn i 20 kobiet) i pacjentów z MS (20 mężczyzn i 20 kobiet). W gru-pach badanych oznaczono w surowicy stężenie parathormonu (PTH), 25-OH-D3, C-końcowego usieciowanego peptydu kolagenu typu 1 (CTX1), osteokalcyny, FGF23, wapnia (Ca) i fosforu (P). Ryzyko złamania osteoporotycznego oszacowano, stosując skalę FRAX (Fracture Risk Assessmen Tool). Grupę kontrolną stanowiło 15 zdrowych mężczyzn i kobiet w podobnym wieku.WynikiOtyłe kobiety z MS charakteryzowały się większym ryzykiem złamań osteoporotycznych (3,0 vs 1,6%; p < 0,001), stężeniem w surowicy fosforu (1,8 vs 1,12 mmol/l; p < 0,001) oraz mniejszym stężeniem 25-OH-D3 (7,3 vs 34,6 ng/ml; p < 0,01) w porównaniu z kobietami bez MS. Nie stwierdzono istotnych różnic w ryzyku złamań osteoporotycznych i stężeniach ocenianych parametrów między mężczyznami z otyłością bez MS i z MS. Kobiety z MS miały niższe stężenie CTX-1 (0,27 ng/ml vs. 0,41 ng/ml; p) w porównaniu z mężczyznami.WnioskiZespół metaboliczny nie wpływa na wskaźniki metabolizmu kostnego u mężczyzn i kobiet. Kobiety z zespołem metabolicznym charakteryzują się jednak mniejszym stężeniem 25-OH-D3. Kobiety, ale nie mężczyźni, z zespołem metabolicznym, wykazują większe 10-letnie ryzyko złamania osteoporotycznego niż kobiety z otyłością bez zespołu metabolicznego.

WstępHiponatremię uważa się za czynnik ryzyka udaru mózgu.Materiał i metodyPrzeanalizowano 677 chorych z pierwszym w życiu udarem niedokrwiennym mózgu, których podzielono w zależności od stężenia sodu w surowicy na dwie podgrupy: ≤ 135 mmol/l i > 135 mmol/l.WynikiU chorych ze stężeniem sodu ≤ 135 mmol/l mediana w skali NIHSS wyniosła 5 pkt, mediana oceny stopnia inwalidztwa według skali mRankin wynosiła 4 pkt, a śmiertelność w okresie jednego miesiąca od zachorowania wynosiła 10,5%, w grupie ze stężeniem sodu > 135 mmol/l stwierdzono odpowiednio: 3 pkt, 2 pkt, i 3,4%. U chorych z hiponatremią śmiertelność w ciągu miesiąca od zachorowania wynosiła u kobiet 16,4%, u mężczyzn 3,5% (p = 0,0194). U kobiet z prawidłową natremią obserwowano większy stopień inwalidztwa w skali mRankin (p = 0,005).WnioskiHiponatremia ma niekorzystny wpływ na stan pacjentów zarówno w ostrej, jak i podostrej fazie udaru oraz wiąże się ze zwiększoną śmiertelnością miesięczną od zachorowania. Kobiety z hiponatremią cechowała większa śmiertelność w ciągu jednego miesiąca po przebytym pierwszym w życiu udarze niedokrwiennym. U kobiet z prawidłowym stężeniem sodu w czasie pierwszego w życiu udaru niedokrwiennego stwierdzano wyższy stopień inwalidztwa ocenianego w skali mRankin w porównaniu z mężczyznami.

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

Background Vascultides are a heterogeneous group of rare diseases with unknown etiology and the clinical spectrum ranging from life–threatening systemic disease to minor isolated skin changes. Present nomenclature and definitions of systemic vasculitides have been proposed in 2012 at the International Chapel Hill Consensus Conference. 1 Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) belong to the small to medium-size vessel systemic diseases comprising granulomatosis with polyangitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). Objectives We decided to retrospectively analyse a large cohort of Polish AAV patients coming from referral centres – members of the Scientific Consortium of the Polish Vasculitis Registry (POLVAS). 2,3 Methods We conducted a systematic multicenter retrospective study of adult patients diagnosed with AAV between Jan 1990 and Dec 2016 Patients were enrolled to the study by 9 referral centres (14 clinical wards: – rheumatology – 10, nephrology – 2, metabolic diseases – 1, clinical immunology −1) from 7 Voivodships populated by 22,3 mln inhabitants (58,2% of the Polish population). Patient data have been collected according to the uniform protocol in the POLVAS registry. Results Distribution of AAV in the Polish population resembles that of north European cohorts. 4 In POLVAS registry 417 (66.7%) patients were diagnosed with GPA, 106 (17.0%) with MPA and 102 (16,3%) with EGPA. Male-to-female ratios were almost 1:1 for GPA (210/207) and MPA (54/52), but EGPA wasd twice more frequent among women (34/68). Clinical manifestations and organ involvement were analysed both by clinical phenotype (GPA, MPA, EGPA) and ANCA specificity (anti-PR3+, and anti-MPO+)Analysis by ANCA specificity showed no apparent difference as compared to phenotype analysis. Clinical manifestations of different AAV in the polish population seems very similar to other European countries. Conclusions This is the first multicenter retrospective study of the Polish patients sufferng from with AAV. Demographic characteristics and disease manifestations of AAV patients in POLVAS registry follow the same pattern as those from other European countries. 4 References [1] Jennette JC, et al. Arthritis Rheum2013Jan;65(1):1–11. [2] Padjas A, et al. Pol Arch Med Wewn2014;124(3):143–4. [3] Musiax J, Wojcik K. Pol Arch Intern Med2017Jan 31;127(1):71–72. [4] Bajema, et al. Kidney Int Rep2017;1018–1031. Disclosure of Interest None declared

BACKGROUND Chronic kidney disease (CKD) is an independent risk factor for cardiovascular morbidity and mortality. Hyponatremia is the most common electrolyte disturbance encountered in the neurological and neurosurgical intensive care units, and can exacerbate existing neurological deficits. The objective of this study was to observe the influences of chronic kidney disease and sodium disturbances on the clinical course of acute and sub-acute stages of first-ever ischemic stroke. MATERIAL AND METHODS 464 patients with previously diagnosed chronic kidney disease (aged 70.42±11.49 years; 250 women) who had experienced their first-ever ischemic stroke were qualified. The following examinations were performed: serum levels of sodium, creatinine, lipids, estimated glomerular filtration rate (eGFR), neurological state on 1st day of stroke (according to National Institutes of Health Stroke Scale), functional state measured with the Rankin scale, (RS) and mortality rate at 1 month after stroke. RESULTS The neurological state on 1st day of stroke was worse and the median RS (30 days after stroke) was higher in patients with eGFR ≤60 ml/ (min×1.73). Men with eGFR ≤60 ml had greater neurological deficits and increased mortality within 1 month. In patients with eGFR >60 ml/, male sex was more often associated with worse outcomes at 1 month after ischemic stroke. Hyponatremia was associated with a more severe state in both the acute and sub-acute stages of stroke, with higher incidence of death within 1 month after stroke. Men with hyponatremia had greater neurological deficits on the 1st day and increased mortality within 1 month. CONCLUSIONS Renal impairment and hyponatremia are associated with worse neurological outcomes in patients in the acute stage of their first-ever stroke and within 1 month after the event. Males with impaired kidney function and hyponatremia have a more severe course in their first-ever ischemic stroke, as well as having increased mortality.

ANCA-associated vasculitides (AAV) are a heterogeneous group of rare diseases with unknown aetiology 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) seem to be clinically much more uniform. Recently, three subcategories of AAV have been proposed and described as non-severe AAV, severe PR3-AAV, and severe MPO-AAV.In line with these attempts, we decided to use an unbiased approach offered by latent class analysis (LCA) to subcategorise GPA and MPA in a large cohort of Polish AAV patients included in a multicentre POLVAS registry.LCA of our AAV group identified a four-class model of AAV, including previously proposed three subphenotypes and revealing a fourth (previously not described) clinically relevant subphenotype. This new subphenotype includes only GPA patients, usually diagnosed at a younger age as compared to other groups, and characterised by multiorgan involvement, high relapse rate, relatively high risk of death, but no end-stage kidney disease.Based on multiple clinical and serological variables, LCA methodology identified 4-class model of AAV. This newly described fourth class of AAV may be of clinical relevance and may require prompt diagnosis and aggressive treatment due to the multiorgan involvement, high risk of relapse and marked mortality among these relatively young GPA subjects.