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Vasculitides in ICU

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36 papers 0 to 25 followers
Georgina Espígol-Frigolé, Sergio Prieto-González, Marco A Alba, Itziar Tavera-Bahillo, Ana García-Martínez, Rosa Gilabert, José Hernández-Rodríguez, Maria C Cid
The diagnosis of large-vessel vasculitis has experienced substantial improvement in recent years. While Takayasu arteritis diagnosis relies on imaging, the involvement of epicranial arteries by giant-cell arteritis facilitates histopathological confirmation. When appropriately performed temporal artery biopsy has high sensitivity and specificity. However, an optimal biopsy is not always achievable and, occasionally, the superficial temporal artery may not be involved. Imaging in its various modalities including colour-duplex ultrasonography, computed tomography angiography, magnetic resonance angiography and positron emission tomography, are emerging as important procedures for the diagnosis and assessment of disease extent in large-vessel vasculitis...
2015: Rheumatic Diseases Clinics of North America
Eli M Miloslavsky, John H Stone, Sebastian H Unizony
The need to distinguish true primary systemic vasculitis from its multiple potential mimickers is one of the most challenging diagnostic conundrums in clinical medicine. This article reviews 9 challenging vasculitis mimickers: fibromuscular dysplasia, calciphylaxis, segmental arterial mediolysis, antiphospholipid syndrome, hypereosinophilic syndrome, lymphomatoid granulomatosis, malignant atrophic papulosis, livedoid vasculopathy, and immunoglobulin G4-related disease.
2015: Rheumatic Diseases Clinics of North America
J S F Sanders, W H Abdulahad, C A Stegeman, C G M Kallenberg
Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) are autoimmune diseases in which the small vessels are inflamed. Clinical observations suggest a pathogenic role for ANCA. Such a role is supported by in vitro experimental data and animal models, particularly for myeloperoxidase-ANCA. An in vivo pathogenic role of ANCA directed to proteinase 3 has, however, not been fully substantiated. Additionally, the pathogenic role of B cells, T cells, and the alternative pathway of complement in AAV have been elucidated...
2014: Nephron. Clinical Practice
Cees G M Kallenberg, Peter Heeringa, Coen A Stegeman
Wegener's granulomatosis and microscopic polyangiitis are idiopathic systemic vasculitides strongly associated with antineutrophil cytoplasmic autoantibodies (ANCA). In Wegener's granulomatosis, ANCA are mostly directed against proteinase 3 (PR3), whereas in microscopic polyangiitis ANCA are directed against myeloperoxidase; increases in levels of these autoantibodies precede or coincide with clinical relapses in many cases. In vitro, ANCA can further activate primed neutrophils to release reactive oxygen species and lytic enzymes, and, in conjunction with neutrophils, can damage and lyse endothelial cells...
December 2006: Nature Clinical Practice. Rheumatology
Wei Ge, Hai-Lian Wang, Ruo-Peng Sun
UNLABELLED: We investigated the potential role of pentraxin 3 (PTX3) in Henoch-Schönlein purpura (HSP), a common multisystemic vasculitis affecting children, as a predictor of Henoch-Schönlein purpura nephritis (HSPN). A total of 108 cases consisting of 34 children with HSP, 37 children with HSPN, and 37 healthy control children were enrolled in this prospective study from March 2010 to February 2013. Blood and urine samples were collected to measure plasma PTX3, C-reactive protein (CRP), serum creatinine, blood urea nitrogen (BUN), urine microalbumin (MALB), and β2-microglobulin (β2-MG)...
February 2014: European Journal of Pediatrics
K Polzer, T Karonitsch, T Neumann, G Eger, C Haberler, A Soleiman, B Hellmich, E Csernok, J Distler, B Manger, K Redlich, G Schett, J Zwerina
OBJECTIVE: Churg-Strauss Syndrome (CSS) is characterized by excessive eosinophil accumulation in peripheral blood and affected tissues with development of granulomatous vasculitic organ damage. The contribution of eosinophil-chemotactic cytokines (eotaxin family) to eosinophilia and disease activity in CSS is unknown. Thus, we compared serum levels of the eotaxin family members in CSS patients with healthy and disease controls. METHODS: Forty patients with CSS diagnosed according to ACR 1990 criteria, 30 healthy controls (HC) and 57 disease controls (28 asthma, 20 small vessel vasculitis, 9 hypereosinophilic syndrome) were studied...
June 2008: Rheumatology
Raashid Ahmed Luqmani
Anti-neutrophil cytoplasm antibodies (ANCA) are associated with small vessel vasculitides (AASV) affecting the lungs and kidneys. Structured clinical assessment using the Birmingham Vasculitis Activity Score and Vasculitis Damage Index should form the basis of a treatment plan and be used to document progress, including relapse. Severe disease with organ or life threatening manifestations needs cyclophosphamide or rituximab, plus high dose glucocorticoids, followed by lower dose steroid plus azathioprine, or methotrexate...
2014: Frontiers in Immunology
Paul A Monach, Philipp Kümpers, Alexander Lukasz, Gunnar Tomasson, Ulrich Specks, John H Stone, David Cuthbertson, Jeffrey Krischer, Simon Carette, Linna Ding, Gary S Hoffman, David Iklé, Cees G M Kallenberg, Nader A Khalidi, Carol A Langford, Philip Seo, E William St Clair, Robert Spiera, Nadia Tchao, Steven R Ytterberg, Marion Haubitz, Peter A Merkel
The endothelial-specific Angiopoietin-Tie2 ligand-receptor system is an important regulator of endothelial activation. Binding of angiopoietin-2 (Ang-2) to Tie2 receptor renders the endothelial barrier responsive to pro-inflammatory cytokines. We previously showed that circulating Ang-2 correlated with disease severity in a small cohort of critically ill patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis. The current study reassessed Ang-2 as a biomarker of disease activity and relapse in AAV...
2012: PloS One
Tania Cellucci, Pascal N Tyrrell, Eleanor Pullenayegum, Susanne M Benseler
OBJECTIVE: The main objective was to develop a trajectory for von Willebrand factor (vWF) antigen in childhood primary CNS vasculitis (cPACNS) after treatment and compare this with disease activity and other inflammatory markers. METHODS: A single-centre cohort study of consecutive children diagnosed with cPACNS was performed. Demographic, clinical, laboratory, imaging and histological data were collected at diagnosis and during standardized clinic visits. Outcome measures included disease activity measured by physician global assessment and serial measures of vWF antigen...
October 2012: Rheumatology
Alexandre de Souza, Johanna Westra, Johan Bijzet, Pieter C Limburg, Coen A Stegeman, Marc Bijl, Cees G M Kallenberg
BACKGROUND: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are systemic inflammatory disorders that include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), Churg-Strauss syndrome and renal limited vasculitis (RLV). Extracellular high-mobility group box 1 (HMGB1) acts as an alarmin and has been shown to be a biomarker of disease activity as well as an autoantigen in systemic lupus erythematosus (SLE) and, possibly, in AAV. This study aims to assess antibodies against HMGB1 and HMGB1 levels as biomarkers for AAV disease activity and predictors of relapsing disease...
2013: Arthritis Research & Therapy
Lindsay Lally, Robert F Spiera
Despite recent advances in the treatment of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV), relapse remains common and patients often experience a variable clinical course after initial treatment. New biomarkers are needed to aid the management of these complex diseases. Discoveries regarding the pathogenesis of AAV, from the importance both of activated B and T cells and the alternative complement pathway to genomic data, may lay the groundwork for identification of novel biomarkers...
October 2013: Current Rheumatology Reports
Ruth J Pepper, Sally Hamour, Konstantia-Maria Chavele, Sarah K Todd, Niels Rasmussen, Shaun Flint, Paul A Lyons, Kenneth G C Smith, Charles D Pusey, H Terence Cook, Alan D Salama
Antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) commonly results in glomerulonephritis, in which neutrophils and monocytes have important roles. The heterodimer calprotectin (S100A8/S100A9, mrp8/14) is a Toll-like receptor-4 ligand found in neutrophils and monocytes and is elevated in inflammatory conditions. By immunohistochemistry of renal biopsies, patients with focal or crescentic glomerular lesions were found to have the highest expression of calprotectin and those with sclerotic the least...
June 2013: Kidney International
Paul A Monach, Gunnar Tomasson, Ulrich Specks, John H Stone, David Cuthbertson, Jeffrey Krischer, Linna Ding, Fernando C Fervenza, Barri J Fessler, Gary S Hoffman, David Ikle, Cees G M Kallenberg, Carol A Langford, Mark Mueller, Philip Seo, E William St Clair, Robert Spiera, Nadia Tchao, Steven R Ytterberg, Yi-Zhong Gu, Ronald D Snyder, Peter A Merkel
OBJECTIVE: To identify biomarkers that distinguish between active antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and remission in a manner superior or complementary to established markers of systemic inflammation. METHODS: Markers of vascular injury and angiogenesis were measured before and after treatment in a large clinical trial in AAV: 163 subjects enrolled in the Rituximab in ANCA-Associated Vasculitis trial were screened for the present study...
December 2011: Arthritis and Rheumatism
Paul A Monach
PURPOSE OF REVIEW: Better biomarkers are needed for guiding management of patients with vasculitis. Large cohorts and technological advances had led to an increase in preclinical studies of potential biomarkers. RECENT FINDINGS: The most interesting markers described recently include a gene expression signature in CD8+ T cells that predicts tendency to relapse or remain relapse-free in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, and a pair of urinary proteins that are elevated in Kawasaki disease but not other febrile illnesses...
January 2014: Current Opinion in Rheumatology
Paul A Monach, Roscoe L Warner, Gunnar Tomasson, Ulrich Specks, John H Stone, Linna Ding, Fernando C Fervenza, Barri J Fessler, Gary S Hoffman, David Iklé, Cees G M Kallenberg, Jeffrey Krischer, Carol A Langford, Mark Mueller, Philip Seo, E William St Clair, Robert Spiera, Nadia Tchao, Steven R Ytterberg, Kent J Johnson, Peter A Merkel
OBJECTIVE: To identify circulating proteins that distinguish between active anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and remission in a manner complementary to markers of systemic inflammation. METHODS: Twenty-eight serum proteins representing diverse aspects of the biology of AAV were measured before and 6 months after treatment in a large clinical trial of AAV. Subjects (n=186) enrolled in the Rituximab in ANCA-Associated Vasculitis (RAVE) trial were studied...
August 2013: Annals of the Rheumatic Diseases
Jason G Lieberthal, David Cuthbertson, Simon Carette, Gary S Hoffman, Nader A Khalidi, Curry L Koening, Carol A Langford, Kathleen Maksimowicz-McKinnon, Philip Seo, Ulrich Specks, Steven R Ytterberg, Peter A Merkel, Paul A Monach
OBJECTIVE: Glomerulonephritis (GN) is common in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), but tools for early detection of renal involvement are imperfect. We investigated 4 urinary proteins as markers of active renal AAV: alpha-1 acid glycoprotein (AGP), kidney injury molecule-1 (KIM-1), monocyte chemoattractant protein-1 (MCP-1), and neutrophil gelatinase-associated lipocalin (NGAL). METHODS: Patients with active renal AAV (n = 20), active nonrenal AAV (n = 16), and AAV in longterm remission (n = 14) were identified within a longitudinal cohort...
May 2013: Journal of Rheumatology
Christian Rabe, Beate Appenrodt, Christian Hoff, Santiago Ewig, Hans Ulrich Klehr, Tilman Sauerbruch, Georg Nickenig, Selçuk Tasci
BACKGROUND: The aim of this study was to characterize patients and report outcome of diffuse alveolar hemorrhage (DAH) requiring intensive care unit support. PATIENTS AND METHODS: Thirty-seven patients were identified. Clinical characteristics and outcome were determined by chart review. RESULTS: Eighty-nine percent of patients presented with shortness of breath, 23% with cough, and 3% with hemoptysis. In 9% of patients, a diagnosis of DAH was suspected on admission...
June 2010: Journal of Critical Care
Spyros A Papiris, Effrosyni D Manali, Ioannis Kalomenidis, Giorgios E Kapotsis, Anna Karakatsani, Charis Roussos
The term Pulmonary-renal syndrome refers to the combination of diffuse alveolar haemorrhage and rapidly progressive glomerulonephritis. A variety of mechanisms such as those involving antiglomerular basement membrane antibodies, antineutrophil cytoplasm antibodies or immunocomplexes and thrombotic microangiopathy are implicated in the pathogenesis of this syndrome. The underlying pulmonary pathology is small-vessel vasculitis involving arterioles, venules and, frequently, alveolar capillaries. The underlying renal pathology is a form of focal proliferative glomerulonephritis...
2007: Critical Care: the Official Journal of the Critical Care Forum
J W Cohen Tervaert
Most frequent reasons for intensive care unit (ICU) admission in vasculitis patients are severe respiratory insufficiency due to diffuse alveolar haemorrhage, sepsis and/or pneumonia and an acute abdomen due to bowel infarction. Other reasons are massive gastrointestinal bleeding, thromboembolism and/or scissures. In a patient, not previously diagnosed as having vasculitis, diagnosis can be difficult and must be made as soon as possible, since immunosuppressive therapy should be instituted immediately. Immunosuppressive therapy in severe cases consists of high-dose corticosteroids and cyclophosphamide...
2007: Acta Clinica Belgica
S Anjum Khan, Mir Rauf Subla, Deepti Behl, Ulrich Specks, Bekele Afessa
PURPOSES: This study aims to describe the clinical course and prognostic factors of patients with small-vessel vasculitis admitted to a medical ICU. METHODS: We reviewed the clinical records of 38 patients with small-vessel vasculitis admitted consecutively to the ICU between January 1997 and May 2004. The APACHE (acute physiology and chronic health evaluation) III prognostic system was used to determine the severity of illness on the first ICU day; the sequential organ failure assessment (SOFA) score was used to measure organ dysfunction, and the Birmingham vasculitis activity score for Wegener granulomatosis (BVAS/WG) was used to assess vasculitis activity...
April 2007: Chest
2014-11-21 19:46:49
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