A5017365560- Vasculitis and related conditions
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Renal Diseases and Glomerulopathies
- Sarcoidosis and Beryllium Toxicity Research
- Otitis Media and Relapsing Polychondritis
- Heparin-Induced Thrombocytopenia and Thrombosis
- Autoimmune and Inflammatory Disorders
- Autoimmune Bullous Skin Diseases
- Amyloidosis: Diagnosis, Treatment, Outcomes
- Systemic Sclerosis and Related Diseases
- Cell Adhesion Molecules Research
- Complement system in diseases
- Immunodeficiency and Autoimmune Disorders
- Urticaria and Related Conditions
- SARS-CoV-2 and COVID-19 Research
- Mast cells and histamine
- COVID-19 Clinical Research Studies
- IgG4-Related and Inflammatory Diseases
- Drug-Induced Adverse Reactions
- Acute Kidney Injury Research
- Chronic Kidney Disease and Diabetes
- Systemic Lupus Erythematosus Research
- Dialysis and Renal Disease Management
- Blood disorders and treatments
- Neutrophil, Myeloperoxidase and Oxidative Mechanisms
Massachusetts General Hospital
2014-2024
Harvard University
1994-2024
GTx (United States)
1999-2013
Boston University
2013
University of California, San Francisco
2001
Harvard University Press
1989-1998
Boston Children's Hospital
1998
Lund University
1997
Jackson Laboratory
1997
Good Samaritan Medical Center
1961
To refine and validate the Birmingham Vasculitis Activity Score (BVAS) as a disease-specific activity index for Wegener's granulomatosis (WG).Sixteen members of International Network Study Systemic Vasculitides (INSSYS) revised BVAS, with 3 goals: to reduce redundancy some component items, enhance its ability capture important disease manifestations specific WG, streamline instrument use in clinical research. We defined items weighted them empirically either minor (e.g., nasal crusting = 1...
Anti-neutrophil cytoplasmic antibody–associated (ANCA-associated) small vessel necrotizing vasculitis is caused by immune-mediated inflammation of the wall and diagnosed in some cases presence myeloperoxidase-specific antibodies (MPO-ANCA). This multicenter study sought to determine whether differences ANCA epitope specificity explain why, cases, conventional serologic assays do not correlate with disease activity, why naturally occurring anti-MPO autoantibodies can exist disease-free...
In the 330-patient ADVOCATE trial of avacopan for treatment antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, in which 81% patients had renal involvement, estimated glomerular filtration rate (eGFR) increased on average 7.3 ml/min per 1.73 m2 group and 4.1 prednisone (P = 0.029) at week 52. This new analysis examines results patient subgroup with severe insufficiency enrollment into trial, i.e., eGFR ≤20 m2.eGFR was determined baseline over course trial. Changes were...
The triggers that induce antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis (APV) are largely unknown. However, there have been reports suggesting hydralazine, propylthiouracil, and several other drugs may cause some cases of APV, the majority these associated with antimyeloperoxidase (anti-MPO) ANCA. Our experience led us to hypothesize high titers anti-MPO antibodies often drug-associated.In this study, we determined prevalence exposure previously implicated in APV among 30...
The pathogenesis of Heymann nephritis, a rat model human membranous glomerulonephritis, depends on the interaction autoantibodies with renal glycoprotein (GP330) glomerular podocytes. Partial complementary DNAs coding for GP330 were isolated and sequenced. deduced amino acid sequence from 4.3 kilobases DNA contains sequences identical to two peptides derived glycoprotein. this protein regions homology low density lipoprotein (LDL) receptor, an indication that LDL receptor may be members same...
In the absence of evidence arteritis or Wegener's granulomatosis, syndrome lung hemorrhage and nephritis has been commonly associated with anti-glomerular basement membrane (GBM) antibodies. However, it increasingly recognized that many cases are antineutrophil cytoplasmic antibodies (ANCAs).To review available clinical pathologic findings to determine diseases accounting for nephritis.We studied records 750 patients from whom serum samples were sent our laboratory anti-GBM antibody assays...
Approximately 70% of illicit cocaine consumed in the United States is contaminated with levamisole. Most commonly used as a veterinary antihelminthic agent, levamisole known immunomodulating agent. Prolonged use humans has been associated cutaneous vasculitis and agranulocytosis. We describe development systemic autoimmune disease antineutrophil cytoplasmic antibodies (ANCA) users. This complication appears to be linked combined exposure.Cases were identified between March 2009 November 2010...
Lysosomal membrane protein 2 (LAMP-2) is a target of antineutrophil cytoplasmic autoantibodies (ANCA) in addition to the more commonly known targets proteinase 3 and myeloperoxidase. The prevalence anti–LAMP-2 antibodies their relationship disease ANCA glomerulonephritis are not well described. We measured reactivity 680 sera samples (two academic centers) from patients with (n=329); those ANCA-negative (n=104); fimbriated, gram-negative Escherichia coli urinary tract infection controls...
Remission in the majority of ANCA vasculitis patients is not sustained after a single course rituximab, and risk relapse warrants development successful strategy to ensure durable remission.A retrospective analysis who underwent maintenance therapy using rituximab-induced continuous B-cell depletion for up 7 years was performed. Maintenance with rituximab initiated achieving remission or converting from other prior therapy. Continuous achieved all by scheduled administration every 4 months....
Objective This study aimed to evaluate the safety of avacopan, an orally administered C5a receptor inhibitor, for treatment antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis in addition standard‐of‐care (SOC) with glucocorticoids cyclophosphamide or rituximab. Methods In this randomized 12‐week study, twice daily avacopan (10 mg 30 mg) plus SOC was assessed versus only patients newly diagnosed/relapsing ANCA‐associated vasculitis. Efficacy measurements included 50% greater...
Individuals with primary and pharmacologic B cell deficiencies have high rates of severe disease mortality from coronavirus 2019 (COVID-19), but the immune responses clinical outcomes after acute respiratory syndrome 2 (SARS-CoV-2) infection vaccination yet to be fully defined. Here, we evaluate cellular both SARS-CoV-2 in patients receiving anti-CD20 therapy rituximab (RTX) those low counts due common variable deficiency (CVID) disease. Assessment effector memory CD4 + CD8 T revealed...
IntroductionPost-marketing data on outcomes of avacopan use in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are lacking.MethodsWe performed a multi-center retrospective analysis 92 patients with newly diagnosed or relapsing AAV who received therapy avacopan. The co-primary outcome measures were clinical remission at 26 and 52 weeks.. We descriptive statistics univariate logistic regression to assess predictors remission, respectively.ResultsOf the patients, 21 (23%)...
An appreciable percentage of patients with serum anti-glomerular basement membrane (anti-GBM) antibodies also have antineutrophil cytoplasmic (ANCA), against either myeloperoxidase (MPO-ANCA), or proteinase 3 (PR3-ANCA). In sera without ANCA, the anti-GBM been shown to react mainly noncollagenous domain (NC1) Type IV collagen, and especially its alpha chain, 3(IV)NC1. most sera, can be partially blocked by a monoclonal antibody (Mab17) 3(IV)NC1, suggesting that limited region is recognized....
Ongoing randomized trials seek to validate the efficacy of rituximab as an induction agent for anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). However, no studies directly address role maintenance therapy.This retrospective study reports authors' experience with continuous administration in 39 patients complete or partial remission at time initiation. All had least 1 year follow-up, and 20 2 years follow-up.Disease activity, measured by a modified Birmingham...
Remission induction in antineutrophil cytoplasmic autoantibody (ANCA) vasculitis may be complicated by slow response to treatment and toxicity from glucocorticoids. We describe outcomes with a novel remission regimen combining rituximab short course of low-dose, oral cyclophosphamide an accelerated prednisone taper.Patients were included this retrospective study if they had newly diagnosed or relapsing ANCA Birmingham Vasculitis Activity Score for Wegener Granulomatosis (BVAS-WG) ≥3 received...
Abstract Introduction Continuous renal replacement therapy (CRRT) is a widely used but resource-intensive treatment. Despite its broad adoption in intensive care units (ICUs), it remains challenging to identify patients who would be most likely achieve positive outcomes with this and provide realistic prognostic information families. Methods We analyzed prospective cohort of all 863 ICU initiated on CRRT at an academic medical center from 2008 2011 either new-onset acute kidney injury (AKI)...
To evaluate the effect of rituximab on pathogenic autoantibodies and total Ig levels, to identify serious adverse events in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) treated continuous B cell depletion.We conducted a retrospective analysis 239 AAV rituximab-induced depletion. Two treatment cohorts were analyzed: an induction group (n = 52) maintenance 237). Changes ANCA titers levels over time evaluated using mixed-effects models. Risk factors for...
Objective: To evaluate the performance characteristics of direct and capture ELISA for detection PR3-ANCA in Wegener's granulomatosis (WG) international ANCA reference laboratories.