Histopathological subgrouping versus renal risk score for the prediction of end-stage renal disease in ANCA-associated vasculitis

2020 ◽  
Vol 79 (5) ◽  
pp. 675-676 ◽  
Author(s):  
Onay Gercik ◽  
Emre Bilgin ◽  
Dilek Solmaz ◽  
Fulya Cakalagaoglu ◽  
Arzu Saglam ◽  
...  
Keyword(s):  
Renal Disease ◽  
Risk Score ◽  
End Stage Renal Disease ◽  
Anca Associated Vasculitis ◽  
Stage Renal Disease ◽  
End Stage

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

Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis: 2-year results of a randomised trial

2015 ◽  
Vol 74 (6) ◽  
pp. 1178-1182 ◽  
Author(s):  
Rachel B Jones ◽  
Shunsuke Furuta ◽  
Jan Willem Cohen Tervaert ◽  
Thomas Hauser ◽  
Raashid Luqmani ◽  
...  
Keyword(s):  
Renal Disease ◽  
B Cell ◽  
End Stage Renal Disease ◽  
Remission Induction ◽  
Control Group ◽  
Intravenous Cyclophosphamide ◽  
Anca Associated Vasculitis ◽  
Renal Vasculitis ◽  
Stage Renal Disease ◽  
End Stage

ObjectivesThe RITUXVAS trial reported similar remission induction rates and safety between rituximab and cyclophosphamide based regimens for antineutrophil cytoplasm antibody (ANCA)-associated vasculitis at 12 months; however, immunosuppression maintenance requirements and longer-term outcomes after rituximab in ANCA-associated renal vasculitis are unknown.MethodsForty-four patients with newly diagnosed ANCA-associated vasculitis and renal involvement were randomised, 3:1, to glucocorticoids plus either rituximab (375 mg/m2/week×4) with two intravenous cyclophosphamide pulses (n=33, rituximab group), or intravenous cyclophosphamide for 3–6 months followed by azathioprine (n=11, control group).ResultsThe primary end point at 24 months was a composite of death, end-stage renal disease and relapse, which occurred in 14/33 in the rituximab group (42%) and 4/11 in the control group (36%) (p=1.00). After remission induction treatment all patients in the rituximab group achieved complete B cell depletion and during subsequent follow-up, 23/33 (70%) had B cell return. Relapses occurred in seven in the rituximab group (21%) and two in the control group (18%) (p=1.00). All relapses in the rituximab group occurred after B cell return.ConclusionsAt 24 months, rates of the composite outcome of death, end-stage renal disease and relapse did not differ between groups. In the rituximab group, B cell return was associated with relapse.Trial registration numberISRCTN28528813.

scholarly journals End-stage renal disease in ANCA-associated vasculitis

2016 ◽  
pp. gfw046 ◽  
Author(s):  
Sergey Moiseev ◽  
Pavel Novikov ◽  
David Jayne ◽  
Nikolay Mukhin
Keyword(s):  
Renal Disease ◽  
End Stage Renal Disease ◽  
Anca Associated Vasculitis ◽  
Stage Renal Disease ◽  
End Stage

scholarly journals POS1428 VALIDATION OF ANCA-ASSOCIATED VASCULITIS AS THE CAUSE OF END-STAGE RENAL DISEASE IN THE UNITED STATES RENAL DATA SYSTEM

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 997.2-998
Author(s):  
C. Cook ◽  
H. Choi ◽  
Z. Wallace
Keyword(s):  
United States ◽  
Renal Disease ◽  
Renal Biopsy ◽  
End Stage Renal Disease ◽  
Medical Records ◽  
The United States ◽  
Diagnosis Code ◽  
Anca Associated Vasculitis ◽  
Stage Renal Disease ◽  
End Stage

Background:Glomerulonephritis and other renal manifestations are common in ANCA-associated vasculitis (AAV). Renal involvement in AAV is associated with adverse outcomes, including end-stage renal disease (ESRD) in up to 25% of patients (1). The United States Renal Data System (USRDS), a national registry of ESRD patients, represents a unique nationwide data source for studying AAV patients with ESRD. Prior research has assessed how often patients with ESRD attributed to AAV have biopsy-proven glomerulonephritis in USRDS (2), but the validity of the diagnosis of AAV as the cause of ESRD in the USRDS remains unknown.Objectives:We aim to validate the diagnosis of AAV as the primary cause of ESRD listed in USRDS.Methods:We identified all patients in the Mass General Brigham (MGB) healthcare system with a billing code for advanced chronic kidney disease or end-stage renal disease or procedure code for dialysis or renal transplantation. We identified all MGB patients fulfilling these criteria to records in the USRDS by name, sex, date of birth, and social security number. From this cohort of patients, we identified those with AAV or related diagnoses listed as the primary disease causing ESRD (ICD9: 446.0, 446.4 or ICD10: M31.3X, M31.7). Two authors reviewed medical records to collect information on whether or not a physician had diagnosed AAV, details of AAV history, renal and non-renal biopsies, and antineutrophil cytoplasmic antibody (ANCA) tests. Discrepancies were resolved through consensus. Details regarding initial ESRD onset date were obtained from the USRDS. To calculate the positive predictive value (PPV) for AAV as the primary cause of ESRD a definite physician diagnosis of AAV (a diagnosis confirmed by two physicians based on available data) in the MGB medical record was used as the gold standard. To calculate sensitivity, we linked the Partners (MGB) AAV Cohort to USRDS records using the same methods. A diagnosis code of AAV as the cause of ESRD was considered a true positive and a diagnosis code for other types of nephritis was considered a false negative.Results:We identified 89 USRDS records linked to MGB medical records in which the primary cause of ESRD was attributed to AAV. Of these, 85 were confirmed to be true cases of AAV after medical record review (PPV=96%) (Table 1). Among the cases classified as AAV, 84 (99%) had a positive ANCA test, which was predominantly MPO/P-ANCA (47, 55%); 36 (42%) had a renal biopsy, all of which were supportive of the diagnosis. The majority of cases were identified as AAV by ICD9 or 10 codes for Wegener’s granulomatosis (446.4 or M313.1). Within the Partners (MGB) AAV cohort linked to USRDS records, 33 (55%) of 60 identified cases had AAV listed as the cause of ESRD; in the remainder, ESRD was attributed to non-specific nephritis codes.Table 1.AAV and non-AAV patients in the USRDS with ESRD due to AAV (N=89)Physician-Diagnosed AAV(N=85)ANCA type n (%)84 (98.8)MPO/P-ANCA+47 (55.3)PR3/C-ANCA+33 (38.8)Renal biopsy n (%)36 (42.4)Pauci-Immune Glomerulonephritis n (%)16 (44%)Non-renal biopsy n (%) Yes10 (11.8) No74 (87.1)Years from AAV diagnosis to ESRD median [IQR]1 [0, 6]Principal diagnosis code (ICD9/ICD10) n (%) Wegener’s granulomatosis (446.4, 446.4B, or M313.1)81 (95.3)Conclusion:We found that the diagnosis of AAV as the primary cause of ESRD in the USRD had a high PPV, suggesting accurate classification of ESRD due to AAV in the USRDS, but that sensitivity was moderate. These findings support the past and future use of the USRDS for research with ESRD attributed to AAV.References:[1]Moiseev S, Novikov P, Jayne D, Mukhin N. End-stage renal disease in ANCA-associated vasculitis. Nephrol Dial Transplant. 2017;32(2):248-53.[2]Layton JB, Hogan SL, Jennette CE, Kenderes B, Krisher J, Jennette JC, et al. Discrepancy between Medical Evidence Form 2728 and renal biopsy for glomerular diseases. Clin J Am Soc Nephrol. 2010;5(11):2046-52.Disclosure of Interests:None declared

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