scholarly journals Relationship Between Markers of Platelet Activation and Inflammation with Disease Activity in Wegener’s Granulomatosis

2011 ◽  
Vol 38 (6) ◽  
pp. 1048-1054 ◽  
Author(s):  
GUNNAR TOMASSON ◽  
MICHAEL LAVALLEY ◽  
KAHRAMAN TANRIVERDI ◽  
JAVIER D. FINKIELMAN ◽  
JOHN C. DAVIS ◽  
...  
Keyword(s):  
Disease Activity ◽  
Platelet Activation ◽  
Wegener’S Granulomatosis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Cd40 Ligand ◽  
Wegener's Granulomatosis ◽  
Soluble Cd40 Ligand ◽  
Monocyte Chemoattractant Protein 1 ◽  
Unit Increase ◽  
Active Disease

Objective.There remains a need for biomarkers to guide therapy in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Our objective was to determine whether measures of platelet activation or inflammation are associated with disease activity in Wegener’s granulomatosis (WG).Methods.Study subjects were participants in a clinical trial. Soluble CD40 ligand (sCD40L), C-reactive protein, interleukin 6 (IL-6), IL-8, monocyte chemoattractant protein 1 (MCP-1), P-selectin, vascular endothelial growth factor, and proteinase 3 (PR3)-specific ANCA were measured by ELISA using plasma samples obtained at baseline (active disease), at remission, and prior to, during, and after first flares. Disease activity was assessed by the Birmingham Vasculitis Activity Score for WG (BVAS/WG). Association of biomarkers with disease activity was determined with conditional logistic and linear regression.Results.Over a mean followup of 27 months, 180 subjects underwent 2044 visits; markers were measured in 563 samples. Longitudinally, all markers other than IL-6 were associated with disease activity. The strongest associations for active disease at baseline versus remission were observed for sCD40L (OR 4.72, 95% CI 2.47–9.03), P-selectin (OR 6.26, 95% CI 2.78–14.10), PR3-ANCA (OR 9.41, 4.03–21.99), and inversely for MCP-1 (OR 0.36, 95% CI 0.22–0.57). BVAS/WG increased by 0.80 (95% CI 0.44–1.16), 0.83 (95% CI 0.42–1.25), and 0.81 (95% CI 0.48–1.15) per unit-increase in PR3-ANCA, sCD40L, and P-selectin, respectively; and decreased by 1.54 (95% CI 0.96–2.12) per unit-increase in MCP-1.Conclusion.Cytokines arising from within the circulation, including those of platelet activation, correlate with disease activity in WG.

Morbidity and mortality associated with subglottic laryngotracheal stenosis in granulomatosis with polyangiitis (Wegener's granulomatosis): a single-centre experience in the United Kingdom

2014 ◽  
Vol 128 (9) ◽  
pp. 831-837 ◽  
Author(s):  
N P Jordan ◽  
H Verma ◽  
A Siddiqui ◽  
G A Morrison ◽  
D P D'Cruz
Keyword(s):  
United Kingdom ◽  
Wegener’S Granulomatosis ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Wegener's Granulomatosis ◽  
Laryngotracheal Stenosis ◽  
Surgical Interventions ◽  
The United Kingdom ◽  
Single Centre Experience

AbstractObjectives:We aimed to determine the prevalence of symptomatic subglottic laryngotracheal stenosis in patients with granulomatosis with polyangiitis (Wegener's granulomatosis); we also wanted to characterise the clinical outcomes and surgical interventions required, and the relapse rate in our cohort.Methods:We undertook a retrospective clinical review of all granulomatosis with polyangiitis patients with symptomatic subglottic laryngotracheal stenosis attending St Thomas' Hospital, London, United Kingdom.Results:Symptomatic subglottic laryngotracheal stenosis developed in 16 per cent of granulomatosis with polyangiitis patients attending our clinic. The median age of patients at diagnosis was 44 years (range: 34–81 years); 78 per cent of those presenting with subglottic laryngotracheal stenosis were women and 22 per cent were men. All patients were white; 67 per cent of patients were proteinase 3-antineutrophil cytoplasmic antibody-positive and 67 per cent developed relapsing disease requiring repeated surgical intervention. Subglottic laryngotracheal stenosis relapse was not associated with active systemic vasculitis elsewhere.Conclusion:Subglottic laryngotracheal stenosis is an uncommon but significant complication of granulomatosis with polyangiitis. Management of subglottic laryngotracheal stenosis requires a multi-disciplinary approach, with both rheumatological and otolaryngological expertise involved, given the relapsing nature of the disease.

Wegener's granulomatosis presenting as acute systemic vasculitis following 20 years of limited tracheobronchial disease

2009 ◽  
Vol 123 (12) ◽  
pp. 1375-1377 ◽  
Author(s):  
J E Peters ◽  
A D Salama ◽  
P W Ind
Keyword(s):  
World Literature ◽  
Wegener’S Granulomatosis ◽  
Systemic Vasculitis ◽  
Systemic Disease ◽  
Airway Disease ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Wegener's Granulomatosis ◽  
Airway Stenosis ◽  
History Of ◽  
Tracheobronchial Disease

AbstractObjective:We report a patient with a 20-year history of apparently idiopathic airways stenoses, who presented with an antineutrophil cytoplasmic antibody (ANCA) associated, acute, systemic vasculitis with necrotising glomerulonephritis, subsequently diagnosed as Wegener's granulomatosis.Methods:We present a case report and a review of the world literature on airway stenosis in Wegener's granulomatosis.Results:To our knowledge, this is the first report of Wegener's granulomatosis manifesting as local airway disease for such a prolonged period, before transforming into a systemic vasculitis.Conclusions:This case highlights the need for physicians to be alert to the possibility of Wegener's granulomatosis as a cause of apparently idiopathic airway stenosis, and to be aware that systemic disease may occur in very long-standing, limited Wegener's granulomatosis.

scholarly journals Esophageal Involvement in Wegener’s Granulomatosis: A Case Report and Review of the Literature

2000 ◽  
Vol 14 (5) ◽  
pp. 449-451 ◽  
Author(s):  
Glen A Fallows ◽  
Sean F Hamilton ◽  
Douglas S Taylor ◽  
S Bharati Reddy
Keyword(s):  
Case Report ◽  
Gastrointestinal Tract ◽  
Disease Activity ◽  
Intestinal Perforation ◽  
Standard Therapy ◽  
Wegener’S Granulomatosis ◽  
Wegener's Granulomatosis ◽  
Small Vessel Vasculitis ◽  
Review Of The Literature ◽  
Systemic Symptoms

Wegener’s granulomatosis is characterized by a granulomatous arteritis involving the upper and lower respiratory tracts, progressive glomerulonephritis and systemic symptoms attributable to small vessel vasculitis. Although multisystemic manifestations are frequent, involvement of the gastrointestinal tract is uncommon. Cases have been reported of intestinal perforation, ulceration and hemorrhage. A patient whose initial presentation of Wegener’s granulomatosis was odynophagia secondary to esophageal vasculitis is described. Endoscopy revealed multiple punched out ulcerations in the esophagus, which resolved with standard therapy for systemic Wegener’s granulomatosis. There are only two previous reports of symptomatic esophageal vasculitis in patients with Wegener’s granulomatosis. These reports illustrate the need to consider odynophagia as a reflection of disease activity as opposed to complications of immunosuppressive therapy.

scholarly journals ANTINEUTROPHIL CYTOPLASMIC ANTIBODY (C-ANCA) LEVELS IN RELATION TO THE TREATMENT OF WEGENER'S GRANULOMATOSIS

1995 ◽  
Vol 98 (6) ◽  
pp. 974-983,1075 ◽  
Author(s):  
SHIROH MAGUCHI ◽  
MASAHIKO TAKIZAWA ◽  
YUKIKO MIYATAKE ◽  
YUUJI NAKAMARU ◽  
SATOSHI FUKUDA ◽  
...  
Keyword(s):  
Wegener’S Granulomatosis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Wegener's Granulomatosis

scholarly journals Internalization of IgG-Coated Targets Results in Activation and Secretion of Soluble CD40 Ligand and RANTES by Human Platelets

2010 ◽  
Vol 18 (2) ◽  
pp. 210-216 ◽  
Author(s):  
Adam J. Antczak ◽  
Joshua A. Vieth ◽  
Navinderjit Singh ◽  
Randall G. Worth
Keyword(s):  
Platelet Activation ◽  
Cytokine Production ◽  
Cd40 Ligand ◽  
Human Platelets ◽  
Cytochalasin D ◽  
Soluble Cd40 Ligand ◽  
Actin Remodeling ◽  
Inflammatory Reactions ◽  
Coated Particles ◽  
Immunological Reactions

ABSTRACTPlatelets are crucial elements for maintenance of hemostasis. Other functions attributable to platelets are now being appreciated, such as their role in inflammatory reactions and host defense. Platelets have been reported to bind immunological stimuli like IgG complexes, and for nearly 50 years it has been speculated that platelets may participate in immunological reactions. Platelets have been reported to bind and internalize various substances, similar to other leukocytes, such as macrophages and dendritic cells. In the present study, we tested the hypothesis that human platelets can bind and internalize IgG-coated particles, similar to leukocytes. To this end, we observed that interaction with IgG-coated beads resulted in platelet activation (as measured by CD62P expression), internalization of targets, and significant soluble CD40 ligand (sCD40L) and RANTES (regulated uponactivation,normalTcellexpresses andsecreted) secretion. Blocking FcγRIIA with monoclonal antibody (MAb) IV.3 or inhibiting actin remodeling with cytochalasin D inhibited platelet activation, internalization, and cytokine production. These data suggest that platelets are capable of mediating internalization of IgG-coated particles, resulting in platelet activation and release of both sCD40L and RANTES.

scholarly journals Myeloperoxidase-antineutrophil Cytoplasmic Antibodies with Cytoplasmic Fluorescence Pattern

2010 ◽  
Vol 2 (01) ◽  
pp. 042-043 ◽  
Author(s):  
Seema Chhabra ◽  
Ranjana Walker Minz ◽  
Lekha Goyal ◽  
Nidhi Sharma
Keyword(s):  
Indirect Immunofluorescence ◽  
Wegener’S Granulomatosis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Wegener's Granulomatosis ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Fluorescence Pattern ◽  
Myeloperoxidase Antineutrophil Cytoplasmic Antibody

ABSTRACTWe report here two rare cases of myeloperoxidase–antineutrophil cytoplasmic antibody (MPO-ANCA)-positive Wegener’s granulomatosis (limited variant) which deceptively produced a cytoplasmic (C-ANCA) pattern on indirect immunofluorescence.

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