Full Text Journal Articles by
Author Ulrich Specks

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International Consensus on ANCA Testing in Eosinophilic Granulomatosis with Polyangiitis.

Sergey Moiseev, Xavier Bossuyt, Yoshihiro Arimura, Daniel Blockmans, Elena Csernok, Jan Damoiseaux, Giacomo Emmi, Luis Felipe Flores-Suárez, Bernhard Hellmich, David Jayne, J Charles Jennette, Mark A Little, Aladdin J Mohammad, Frank Moosig, Pavel Novikov, Christian Pagnoux, Antonella Radice, Ken-Ei Sada, Mårten Segelmark, Yehuda Shoenfeld, Renato A Sinico, Ulrich Specks, Benjamin Terrier, Athanasios G Tzioufas, Augusto Vaglio, Ming-Hui Zhao, Jan Willem Cohen Tervaert, ,

An international consensus on anti-neutrophil cytoplasm antibodies (ANCA) testing in eosinophilic granulomatosis with polyangiitis (EGPA) is presented. ANCA, specific for myeloperoxidase (MPO), can be detected in 30-35% of EGPA patients. MPO-ANCA should be tested with antigen-specific immunoassays in any patient with eosinophilic asthma and clinical features suggesting EGPA, including constitutional ... Read more >>

Am. J. Respir. Crit. Care Med. (American journal of respiratory and critical care medicine)
[2020, :]

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Rituximab as therapy to induce remission after relapse in ANCA-associated vasculitis.

Rona M Smith, Rachel Bronwen Jones, Ulrich Specks, Simon Bond, Marianna Nodale, Reem Aljayyousi, Jacqueline Andrews, Annette Bruchfeld, Brian Camilleri, Simon Carette, Chee Kay Cheung, Vimal Derebail, Tim Doulton, Lindsy Forbess, Shouichi Fujimoto, Shunsuke Furuta, Ora Gewurz-Singer, Lorraine Harper, Toshiko Ito-Ihara, Nader Khalidi, Rainer Klocke, Curry Koening, Yoshinori Komagata, Carol Langford, Peter Lanyon, Raashid Ahmed Luqmani, Hirofumi Makino, Carole McAlear, Paul Monach, Larry W Moreland, Kim Mynard, Patrick Nachman, Christian Pagnoux, Fiona Pearce, Chen Au Peh, Charles Pusey, Dwarakanathan Ranganathan, Rennie L Rhee, Robert Spiera, Antoine G Sreih, Vladimir Tesar, Giles Walters, Michael H Weisman, Caroline Wroe, Peter Merkel, David Jayne, , ,

OBJECTIVES:Evaluation of rituximab and glucocorticoids as therapy to induce remission after relapse in ANCA-associated vasculitis (AAV) in a prospective observational cohort of patients enrolled into the induction phase of the RITAZAREM trial. METHODS:Patients relapsing with granulomatosis with polyangiitis or microscopic polyangiitis were prospectively enrolled and received remission-induction therapy with rituximab ... Read more >>

Ann. Rheum. Dis. (Annals of the rheumatic diseases)
[2020, :]

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Radiologic and pathologic characteristics of myeloperoxidase-antineutrophil cytoplasmic antibody-associated interstitial lung disease: a retrospective analysis.

Misbah Baqir, Eunhee E Yi, Thomas V Colby, Christian W Cox, Jay H Ryu, Ulrich Specks,

Background:The association between interstitial lung disease (ILD) and myeloperoxidase (MPO)-antineutrophil cytoplasmic antibodies (ANCAs) has been described, but pathologic characteristics are not well characterized. Objectives:We assessed the radiologic and pathologic characteristics of ILD in MPO-ANCA-positive patients and the association between ILD and vasculitis, particularly microscopic polyangiitis (MPA). Methods:We retrospectively searched electronic ... Read more >>

Sarcoidosis Vasc Diffuse Lung Dis (Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG)
[2019, 36(3):195-201]

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Pulmonary Manifestations of Vasculitis.

Ulrich Specks,

Respiratory manifestations in patients with a primary systemic vasculitis syndrome need to be separated into those caused by the vasculitis itself, those related to treatment complications, and those caused by unrelated or only indirectly related comorbidities such as obstructive sleep apnea. Respiratory tract involvement is most common in the ANCA-associated ... Read more >>

(Pulmonary Manifestations of Rheumatic Disease)
[0, :123-137]

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Pulmonary Eosinophilic Granulomatosis with Polyangiitis Has IgG4 Plasma Cells and Immunoregulatory Features.

Zachary M Dong, Edwin Lin, Michael E Wechsler, Peter F Weller, Amy D Klion, Bruce S Bochner, Don A Delker, Mark W Hazel, Keke Fairfax, Paneez Khoury, Praveen Akuthota, Peter A Merkel, Anne-Marie Dyer, Carol Langford, Ulrich Specks, Gerald J Gleich, Vernon M Chinchilli, Benjamin Raby, Mark Yandell, Frederic Clayton,

The immunologic mechanisms promoting eosinophilic granulomatosis with polyangiitis (EGPA) are unclear. To characterize the mechanisms underlying pulmonary EGPA, we examined and compared EGPA paraffin-embedded lung biopsies with normal lung biopsies, using immunostaining, RNA sequencing, and RT-PCR. The results revealed novel type 2 as well as immuneregulatory features. These features included ... Read more >>

Am. J. Pathol. (The American journal of pathology)
[2020, 190(7):1438-1448]

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Non-Linear Rituximab Pharmacokinetics and Complex Relationship between Rituximab Concentrations and Anti-Neutrophil Cytoplasmic Antibodies (ANCA) in ANCA-Associated Vasculitis: The RAVE Trial Revisited.

Amina Bensalem, Denis Mulleman, Gilles Paintaud, Nicolas Azzopardi, Valérie Gouilleux-Gruart, Divi Cornec, Ulrich Specks, David Ternant,

BACKGROUND AND OBJECTIVES:Rituximab is approved in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and leads to a decrease of ANCA levels. The objectives of this study were to investigate the non-linear pharmacokinetics of rituximab and the relationship between its concentrations and ANCA levels in AAV patients. METHODS:Ninety-two AAV patients ... Read more >>

Clin Pharmacokinet (Clinical pharmacokinetics)
[2020, 59(4):519-530]

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Comment on: Subclassifying ANCA-associated vasculitis: a unifying view of disease spectrum: reply.

Alfred Mahr, Ulrich Specks, David Jayne,

Rheumatology (Oxford) (Rheumatology (Oxford, England))
[2020, 59(5):1187]

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Plasma Exchange and Glucocorticoids in Severe ANCA-Associated Vasculitis.

Michael Walsh, Peter A Merkel, Chen-Au Peh, Wladimir M Szpirt, Xavier Puéchal, Shouichi Fujimoto, Carmel M Hawley, Nader Khalidi, Oliver Floßmann, Ron Wald, Louis P Girard, Adeera Levin, Gina Gregorini, Lorraine Harper, William F Clark, Christian Pagnoux, Ulrich Specks, Lucy Smyth, Vladimir Tesar, Toshiko Ito-Ihara, Janak Rashme de Zoysa, Wojciech Szczeklik, Luis Felipe Flores-Suárez, Simon Carette, Loïc Guillevin, Charles D Pusey, Alina L Casian, Biljana Brezina, Andrea Mazzetti, Carol A McAlear, Elizabeth Broadhurst, Donna Reidlinger, Samir Mehta, Natalie Ives, David R W Jayne, ,

BACKGROUND:More effective and safer treatments are needed for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. METHODS:We conducted a randomized trial with a 2-by-2 factorial design to evaluate the use of plasma exchange and two regimens of oral glucocorticoids in patients with severe ANCA-associated vasculitis (defined by an estimated glomerular filtration rate of ... Read more >>

N. Engl. J. Med. (The New England journal of medicine)
[2020, 382(7):622-631]

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Urinary soluble CD163 and monocyte chemoattractant protein-1 in the identification of subtle renal flare in anti-neutrophil cytoplasmic antibody-associated vasculitis.

Sarah M Moran, Paul A Monach, Lina Zgaga, David Cuthbertson, Simon Carette, Nader A Khalidi, Curry L Koening, Carol A Langford, Carol A McAlear, Larry Moreland, Christian Pagnoux, Philip Seo, Ulrich Specks, Antoine Sreih, Jason Wyse, Steven R Ytterberg, Peter A Merkel, Mark A Little, ,

BACKGROUND:Prior work has shown that urinary soluble CD163 (usCD163) displays excellent biomarker characteristics for detection of active renal vasculitis using samples that included new diagnoses with highly active renal disease. This study focused on the use of usCD163 in the detection of the more clinically relevant state of mild renal ... Read more >>

Nephrol. Dial. Transplant. (Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association)
[2020, 35(2):283-291]

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Eosinophilic Granulomatosis With Polyangiitis: Clinical Predictors of Long-term Asthma Severity.

Alvise Berti, Divi Cornec, Marta Casal Moura, Robert J Smyth, Lorenzo Dagna, Ulrich Specks, Karina A Keogh,

BACKGROUND:The long-term clinical course of asthma in patients with eosinophilic granulomatosis with polyangiitis (EGPA) remains unclear. We aimed to characterize long-term asthma in EGPA and to identify baseline predictors of long-term asthma severity. METHODS:This retrospective cohort study included patients who fulfilled standardized criteria for EGPA who were followed up in ... Read more >>

Chest (Chest)
[2020, 157(5):1086-1099]

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Asthma control in eosinophilic granulomatosis with polyangiitis treated with rituximab.

Marta Casal Moura, Alvise Berti, Karina A Keogh, Gerald W Volcheck, Ulrich Specks, Misbah Baqir,

OBJECTIVES:Rituximab (RTX) treatment is used for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, but its benefits in eosinophilic granulomatosis with polyangiitis (EGPA) are unclear. Our aim was to characterize asthma control and glucocorticoid (GC) sparing after RTX treatment. METHODS:A retrospective, computer-assisted search was performed to identify patients with EGPA and GC-dependent asthma ... Read more >>

Clin. Rheumatol. (Clinical rheumatology)
[2020, 39(5):1581-1590]

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Chronic rhinosinusitis in eosinophilic granulomatosis with polyangiitis: clinical presentation and antineutrophil cytoplasmic antibodies.

Christopher M Low, Karina A Keogh, Elias S Saba, Nelson R Gruszczynski, Alvise Berti, Ulrich Specks, Misbah Baqir, Byron M Smith, Garret Choby, Janalee K Stokken, Erin K O'Brien,

BACKGROUND:In this study we aim to describe presenting characteristics and identify prognostic factors for disease resolution in patients with chronic rhinosinusitis (CRS) in the setting of eosinophilic granulomatosis with polyangiitis (EGPA). METHODS:Patients evaluated at a tertiary care center with diagnoses of EGPA and CRS were identified. Descriptive statistics were obtained. ... Read more >>

Int Forum Allergy Rhinol (International forum of allergy & rhinology)
[2020, 10(2):217-222]

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Remission maintenance in ANCA-associated vasculitis: does one size fit all?

Alvise Berti, Ulrich Specks,

Introduction: The majority of the patients with anti-neutrophil cytoplasmic autoantibody (ANCA) associated vasculitis (AAV) achieve remission with effective induction therapy. Therefore, prevention of relapses and avoiding long-term damage and treatment-related toxicity are major challenges.Areas covered: This review provides an update on maintenance therapy in AAV, emphasizing the available treatment options ... Read more >>

Expert Rev Clin Immunol (Expert review of clinical immunology)
[2019, 15(12):1273-1286]

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Subglottic stenosis and endobronchial disease in granulomatosis with polyangiitis.

Kaitlin A Quinn, Alexander Gelbard, Cailin Sibley, Arlene Sirajuddin, Marcela A Ferrada, Marcus Chen, David Cuthbertson, Simon Carette, Nader A Khalidi, Curry L Koening, Carol A Langford, Carol A McAlear, Paul A Monach, Larry W Moreland, Christian Pagnoux, Philip Seo, Ulrich Specks, Antoine G Sreih, Steven R Ytterberg, Peter A Merkel, Peter C Grayson,

OBJECTIVES:To describe tracheobronchial disease in patients with granulomatosis with polyangiitis (GPA) and evaluate the utility of dynamic expiratory CT to detect large-airway disease. METHODS:Demographic and clinical features associated with the presence of subglottic stenosis (SGS) or endobronchial involvement were assessed in a multicentre, observational cohort of patients with GPA. A ... Read more >>

Rheumatology (Oxford) (Rheumatology (Oxford, England))
[2019, 58(12):2203-2211]

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Association of Pulmonary Hemorrhage, Positive Proteinase 3, and Urinary Red Blood Cell Casts With Venous Thromboembolism in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

Andreas Kronbichler, Johannes Leierer, Jae Il Shin, Peter A Merkel, Robert Spiera, Philip Seo, Carol A Langford, Gary S Hoffman, Cees G M Kallenberg, E William St Clair, Paul Brunetta, Fernando C Fervenza, Duvuru Geetha, Karina A Keogh, Paul A Monach, Steven R Ytterberg, Gert Mayer, Ulrich Specks, John H Stone, ,

OBJECTIVE:To assess the frequency of venous thromboembolism (VTE) events in the Rituximab in Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis (RAVE) trial and identify novel potential risk factors. METHODS:VTE events in 197 patients enrolled in the RAVE trial were analyzed. Baseline demographic and clinical characteristics were recorded, and univariate and multivariate analyses ... Read more >>

(Arthritis & rheumatology (Hoboken, N.J.))
[2019, 71(11):1888-1893]

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Subclassifying ANCA-associated vasculitis: a unifying view of disease spectrum.

Alfred Mahr, Ulrich Specks, David Jayne,

Rheumatology (Oxford) (Rheumatology (Oxford, England))
[2019, 58(10):1707-1709]

Cited: 2 times

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Disease Activity, Antineutrophil Cytoplasmic Antibody Type, and Lipid Levels in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

Zachary S Wallace, Xiaoqing Fu, Katherine Liao, Cees G M Kallenberg, Carol A Langford, Peter A Merkel, Paul Monach, Philip Seo, Ulrich Specks, Robert Spiera, E William St Clair, Yuqing Zhang, Hyon Choi, John H Stone,

OBJECTIVE:Patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have an elevated risk of cardiovascular disease (CVD). This study was undertaken to develop a clearer understanding of the association between changes in disease activity and lipid levels in AAV, which may inform CVD risk stratification in this population. METHODS:Lipid levels were ... Read more >>

(Arthritis & rheumatology (Hoboken, N.J.))
[2019, 71(11):1879-1887]

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Evaluation of Potential Serum Biomarkers of Disease Activity in Diverse Forms of Vasculitis.

Alicia Rodriguez-Pla, Roscoe L Warner, David Cuthbertson, Simon Carette, Nader A Khalidi, Curry L Koening, Carol A Langford, Carol A McAlear, Larry W Moreland, Christian Pagnoux, Philip Seo, Ulrich Specks, Antoine G Sreih, Steven R Ytterberg, Kent J Johnson, Peter A Merkel, Paul A Monach, ,

OBJECTIVE:We evaluated potential circulating biomarkers of disease activity in giant cell arteritis (GCA), Takayasu arteritis (TA), polyarteritis nodosa (PAN), and eosinophilic granulomatosis with polyangiitis (EGPA). METHODS:A panel of 22 serum proteins was tested in patients enrolled in the Vasculitis Clinical Research Consortium Longitudinal Studies of GCA, TA, PAN, or EGPA. ... Read more >>

J. Rheumatol. (The Journal of rheumatology)
[2020, 47(7):1001-1010]

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Rituximab Versus Cyclophosphamide for Central Nervous System Involvement of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A Case Series.

Rachana Krishna, Federica De Giacomi, Neeraj Kumar, Peter Kalina, Ulrich Specks, Misbah Baqir,

OBJECTIVE:To compare the effectiveness of rituximab (RTX) with cyclophosphamide (CYC) in patients who have central nervous system (CNS) involvement in antineutrophil cytoplasmic antibody-associated vasculitis (AAV). METHODS:A computer-assisted search was conducted to identify all adults who received a diagnosis of AAV with CNS involvement from January 1, 1997, through July 1, ... Read more >>

J Clin Rheumatol (Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases)
[2019, :]

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Update on the Management of Respiratory Manifestations of the Antineutrophil Cytoplasmic Antibodies-Associated Vasculitides.

Gwen E Thompson, Ulrich Specks,

Antibody-associated vasculitis comprises 3 small vessel vasculitis syndromes: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic polyangiitis (EGPA). This article outlines the major tracheobronchial and pulmonary parenchymal disease manifestations of GPA and MPA and their management, as well as relevant recent advances in the treatment of EGPA. Shared trends ... Read more >>

Clin. Chest Med. (Clinics in chest medicine)
[2019, 40(3):573-582]

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Incidence, prevalence, mortality and chronic renal damage of anti-neutrophil cytoplasmic antibody-associated glomerulonephritis in a 20-year population-based cohort.

Alvise Berti, Emilie Cornec-Le Gall, Divi Cornec, Marta Casal Moura, Eric L Matteson, Cynthia S Crowson, Aishwarya Ravindran, Sanjeev Sethi, Fernando C Fervenza, Ulrich Specks,

BACKGROUND:True population-based clinical and outcomes data are lacking for anti-neutrophil cytoplasmic autoantibody (ANCA)-associated glomerulonephritis (AAGN). Therefore we aimed to estimate the incidence, prevalence and mortality of AAGN, as well as the relationship between the grade of chronic renal damage at presentation and renal and non-renal outcomes. METHODS:Patients with AAGN were ... Read more >>

Nephrol. Dial. Transplant. (Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association)
[2019, 34(9):1508-1517]

Cited: 1 time

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The association of serum interleukin-6 levels with clinical outcomes in antineutrophil cytoplasmic antibody-associated vasculitis.

Alvise Berti, Roscoe Warner, Kent Johnson, Divi Cornec, Darrell R Schroeder, Brian F Kabat, Carol A Langford, Cees G M Kallenberg, Philip Seo, Robert F Spiera, E William St Clair, Fernando C Fervenza, John H Stone, Paul A Monach, Ulrich Specks, Peter A Merkel, ,

OBJECTIVE:To investigate serum IL-6 (sIL-6) levels during active disease, complete remission (CR), and relapse in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), and to explore the association of changes in sIL-6 with clinical outcomes. METHODS:sIL-6 levels were measured at baseline and longitudinally over 18 months, in 78 patients with AAV enrolled ... Read more >>

J. Autoimmun. (Journal of Autoimmunity)
[2019, 105:102302]

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The Incidence of Arterial and Venous Thrombosis in Antineutrophil Cytoplasmic Antibody-associated Vasculitis.

Alvise Berti, Eric L Matteson, Cynthia S Crowson, Ulrich Specks, Divi Cornec,

J. Rheumatol. (The Journal of rheumatology)
[2019, 46(9):1243]

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Exostosin 1/Exostosin 2-Associated Membranous Nephropathy.

Sanjeev Sethi, Benjamin J Madden, Hanna Debiec, M Cristine Charlesworth, LouAnn Gross, Aishwarya Ravindran, Amber M Hummel, Ulrich Specks, Fernando C Fervenza, Pierre Ronco,

BACKGROUND:In membranous nephropathy (MN), which is characterized by deposition of immune complexes along the glomerular basement membrane (GBM), phospholipase A2 receptor (PLA2R) and thrombospondin type 1 domain-containing 7A are target antigens in approximately 70% and 1%-5% of cases of primary MN, respectively. In other cases of primary MN and in ... Read more >>

J. Am. Soc. Nephrol. (Journal of the American Society of Nephrology : JASN)
[2019, 30(6):1123-1136]

Cited: 1 time

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Mutation Effects on Remote Epitopes of An Autoantigen Decoded with Simulated B-Factors

Yuan-Ping Pang, Marta Casal Moura, Gwen Thompson, Amber Hummel, Darlene Nelson, Dieter Jenne, Ulrich Specks,

Proteinase 3 (PR3) is a neutrophil serine protease targeted by anti-neutrophil cytoplasmic antibodies (ANCAs) in the autoimmune disease granulomatosis with polyangiitis (GPA) 1–5 . PR3 mutants were developed to investigate how PR3 interacts with the ANCAs and whether the interactions can be intervened by therapeutics. One mutant with a Ser195Ala ... Read more >>

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