The level of urinary α1 microglobulin excretion is a useful marker of peritubular capillaritis in antineutrophil cytoplasmic antibody associated vasculitis

2014 ◽  
Vol 19 (5) ◽  
pp. 851-858 ◽  
Author(s):  
Naro Ohashi ◽  
Sayaka Ishigaki ◽  
Kazuto Kitajima ◽  
Naoko Tsuji ◽  
Shinsuke Isobe ◽  
...  
2021 ◽  
Vol 23 (6) ◽  
Author(s):  
Martin Windpessl ◽  
Erica L. Bettac ◽  
Philipp Gauckler ◽  
Jae Il Shin ◽  
Duvuru Geetha ◽  
...  

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


2021 ◽  
Vol 10 (7) ◽  
pp. 1538
Author(s):  
Désirée Tampe ◽  
Peter Korsten ◽  
Philipp Ströbel ◽  
Samy Hakroush ◽  
Björn Tampe

Background: Renal involvement is a common and severe complication of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), potentially resulting in a pauci-immune necrotizing and crescentic ANCA glomerulonephritis (GN) with acute kidney injury (AKI), end-stage renal disease (ESRD) or death. There is recent evidence that the degree of proteinuria at diagnosis is associated with long-term renal outcome in ANCA GN. Therefore, we here aimed to systematically describe the association between proteinuria and clinicopathological characteristics in 53 renal biopsies with ANCA GN and corresponding urinary samples at admission. Methods: A total number of 53 urinary samples at admission and corresponding renal biopsies with confirmed renal involvement of AAV were retrospectively included from 2015 to 2021 in a single-center study. Results: Proteinuria correlated with myeloperoxidase (MPO) subtype, diagnosis of microscopic polyangiitis (MPA) and severe deterioration of kidney function. Proteinuria was most prominent in sclerotic class ANCA GN and ANCA renal risk score (ARRS) high risk attributed to nonselective proteinuria, including both glomerular and tubular proteinuria. Finally, there was no association between proteinuria and systemic disease activity, suggesting that proteinuria reflected specific renal involvement in AAV rather that systemic disease activity. Conclusions: In conclusion, proteinuria correlated with distinct clinicopathological characteristics in ANCA GN, mostly attributed to a reduced fraction of normal glomeruli. Furthermore, proteinuria in ANCA GN reflected specific renal involvement in AAV rather than systemic disease activity. Therefore, urinary findings could further improve our understanding of mechanisms promoting kidney injury and progression of ANCA GN.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1885.2-1886
Author(s):  
R. Sakai ◽  
E. Tanaka ◽  
M. Harigai

Background:Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) requires a long-term maintenance therapy (MT), often accompanied by hospitalization due to relapse and/or comorbidities such as infection1. However, data about direct medical costs of hospitalization during MT in patients with AAV is limited to date despite of an increasing concern about the economic burden of patients with AAV2-3.Objectives:To describe frequency of hospitalization and its direct medical costs during MT after the remission-induction therapy (RT) in patients with AAV using Japanese health insurance database.Methods:This retrospective longitudinal population-based study was conducted using claims data in Japan provided by Medical Data Vision Co., Ltd. We defined individuals as AAV cases receiving RT if they met all of the following: 1) having at least one ICD10 code (M300, M301, M313, or M318); 2) having at least one prescription of oral corticosteroids with prednisolone-equivalent dosage ≥30 mg/day, methylprednisolone pulse therapy, immunosuppressive drugs (cyclophosphamide [IVCY], methotrexate, or mycophenolate mofetil), or rituximab (RTX) during hospitalization between April 2008 and April 2017; and 3) having at least 7 days of hospitalization. The observation started from the next day of discharge from the first hospitalization for RT and ended at 24 months later, the month of loss of follow-up, or April 2017. We described the frequency of hospitalization and calculated direct medical costs (per month) during the observation. We analyzed medical costs from a societal perspective. We classified reasons of hospitalization into 3 categories; intensification of treatments for AAV, AAV MT including IVCY or RTX treatments, and comorbidities (infection, cardiovascular disease [CVD], malignancy, and others) using ICD10 codes plus treatments or interventions during the hospitalization.Results:In this study, 1,703 patients with AAV were included. The median [IQR] age was 72 [63, 79] years and 55.7% were female. The total number of hospitalization was 1,897 in 863 patients (50.7%). Among the hospitalizations, 296 hospitalization in 235 patients were categorized as intensification of treatments for AAV, 627 hospitalization in 297 patients were AAV MT, and 974 hospitalization in 572 patients were categorized as comorbidities. In the last category, infections were most frequent (220), followed by malignancy (54) and CVD (15). The mean direct medical costs per month was 20,945 EUR (1 EUR=125 JPY) in patients with hospitalization and 599 EUR in those without. Patients with hospitalization due to intensification of treatments for AAV had the highest direct medical costs (3,000 EUR), followed by those with hospitalization due to comorbidities (2,001 EUR), and those with hospitalization due to AAV MT (1,649 EUR).Conclusion:More than half of the patients had hospitalization during MT, and hospitalization due to comorbidities were most frequent. The mean direct medical costs in patients with at least one hospitalization was approximately 3.5 times as high as that in those without hospitalization.References:[1]Presse Med. 2015; 44:e251-e257[2]J. Rheumatol. 2015; 42:2383-91[3]Clin Exp Rheumatol.2019:137-43Acknowledgments:This work was supported by AMED under Grant Number JP17ek0109121.Disclosure of Interests:Ryoko Sakai Grant/research support from: Tokyo Women’s Medical University (TWMU) has received unrestricted research grants forDivision of Epidemiology and Pharmacoepidemiology of Rheumatic Diseases from Ayumi Pharmaceutical Co. Ltd., Bristol Meyers Squib, Chugai Pharmaceutical Co. Ltd., Nippon Kayaku Co. Ltd., Taisho Toyama Pharmaceutical Co. Ltd., Mitsubishi Tanabe Pharma Corp., and with which TWMU paid the salary of R.S., Eiichi Tanaka Consultant of: ET has received lecture fees or consulting fees from Abbvie, Asahi Kasei pharma co., Bristol Myers Squibb, Chugai Pharmaceutical, Daiichi Sankyo Co., Eisai Pharmaceutical, Janssen Pharmaceutical K.K., Nippon Kayaku, Pfizer, Takeda Pharmaceutical, Taisho Toyama Pharmaceutical Co., and UCB Pharma., Speakers bureau: ET has received lecture fees or consulting fees from Abbvie, Asahi Kasei pharma co., Bristol Myers Squibb, Chugai Pharmaceutical, Daiichi Sankyo Co., Eisai Pharmaceutical, Janssen Pharmaceutical K.K., Nippon Kayaku, Pfizer, Takeda Pharmaceutical, Taisho Toyama Pharmaceutical Co., and UCB Pharma., masayoshi harigai Grant/research support from: AbbVie Japan GK, Ayumi Pharmaceutical Co., Bristol Myers Squibb Co., Ltd., Eisai Co., Ltd., Mitsubishi Tanabe Pharma Co., Nippon Kayaku Co., Ltd., and Teijin Pharma Ltd. MH has received speaker’s fee from AbbVie Japan GK, Ayumi Pharmaceutical Co., Boehringer Ingelheim Japan, Inc., Bristol Myers Squibb Co., Ltd., Chugai Pharmaceutical Co., Ltd., Eisai Co., Ltd., Eli Lilly Japan K.K., GlaxoSmithKline K.K., Kissei Pharmaceutical Co., Ltd., Oxford Immuotec, Pfizer Japan Inc., and Teijin Pharma Ltd. MH is a consultant for AbbVie, Boehringer-ingelheim, Kissei Pharmaceutical Co., Ltd. and Teijin Pharma.


Author(s):  
Krishna Prasad ◽  
Pruthvi C Revaiah ◽  
Krishna Santosh Vemuri ◽  
Parag Barwad

Abstract Background Antineutrophil cytoplasmic antibody (ANCA)-associated pulmonary renal vasculitis is an uncommon disease entity. Its presentation as acute heart failure for the first time in a patient with established coronary artery disease (CAD) is even rarer. We present here a case of such an association and an approach to managing this clinical situation. Case summary A 60-year-old male patient presented to the emergency room with recent-onset dyspnoea New York Heart Association Class IV. He was having hypertension, uncontrolled diabetes mellitus, chronic kidney disease (CKD), and CAD. He also underwent a percutaneous coronary intervention to left anterior descending in the past for acute coronary syndrome and had moderate left ventricular dysfunction. He was being managed as a case of acute decompensated heart failure (ADHF) and was mechanically ventilated. Suddenly his ventilator requirement increased and endotracheal aspirate contained blood. The chest radiograph showed bilateral hilar infiltrates. Simultaneously he also had recurrent episodes of ventricular tachycardia (VT) requiring direct current (DC) cardioversion. Blood investigations showed deranged renal function and severe hyperkalaemia, but no evidence of coagulopathy. High-resolution computed tomography chest showed features of diffuse alveolar haemorrhage. Further investigations revealed high titres of c-ANCA and raised inflammatory biomarkers. A diagnosis of ANCA-associated vasculitis presenting as acute on CKD with dyselectrolytaemia (hyperkalaemia) leading to VT was made. Apart from standard management for associated illness, he was treated with plasma exchange, steroids, and cyclophosphamide to which he responded and was later on discharged. Discussion Antineutrophil cytoplasmic antibody-related pulmonary renal vasculitis can lead to rapidly progressing renal failure and may present as ADHF in a patient with existent CAD. The associated VT storm in our patient can be attributed to hyperkalaemia secondary to acute renal failure. A multidisciplinary approach is required for the successful management of such a complex clinical scenario.


2019 ◽  
Vol 46 (9) ◽  
pp. 1243-1243 ◽  
Author(s):  
ALVISE BERTI ◽  
ERIC L. MATTESON ◽  
CYNTHIA S. CROWSON ◽  
ULRICH SPECKS ◽  
DIVI CORNEC

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