Relationship between antineutrophil cytoplasmic antibody levels and disease activity in wegener's granulomatosis: comment on article by kerr et al

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.

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Image analysis: a novel approach for the quantification of antineutrophil cytoplasmic antibody levels in patients with Wegener's granulomatosis

Journal of Immunological Methods ◽

10.1016/s0022-1759(02)00273-9 ◽

2003 ◽

Vol 274 (1-2) ◽

pp. 27-35 ◽

Cited By ~ 26

Author(s):

Maarten M Boomsma ◽

Jan G.M.C Damoiseaux ◽

Coen A Stegeman ◽

Cees G.M Kallenberg ◽

Meeta Patnaik ◽

...

Keyword(s):

Image Analysis ◽

Wegener’S Granulomatosis ◽

Antineutrophil Cytoplasmic Antibody ◽

Wegener's Granulomatosis ◽

Novel Approach ◽

Antibody Levels

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Prediction of relapses in Wegener's granulomatosis by measurement of antineutrophil cytoplasmic antibody levels: A prospective study

Arthritis & Rheumatism ◽

10.1002/1529-0131(200009)43:9<2025::aid-anr13>3.0.co;2-o ◽

2000 ◽

Vol 43 (9) ◽

pp. 2025-2033 ◽

Cited By ~ 286

Author(s):

M. M. Boomsma ◽

C. A. Stegeman ◽

M. J. Van Der Leij ◽

W. Oost ◽

J. Hermans ◽

...

Keyword(s):

Prospective Study ◽

Wegener’S Granulomatosis ◽

Antineutrophil Cytoplasmic Antibody ◽

Wegener's Granulomatosis ◽

A Prospective Study ◽

Antibody Levels

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

The Journal of Laryngology & Otology ◽

10.1017/s0022215114001650 ◽

2014 ◽

Vol 128 (9) ◽

pp. 831-837 ◽

Cited By ~ 5

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.

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

The Journal of Laryngology & Otology ◽

10.1017/s002221510900454x ◽

2009 ◽

Vol 123 (12) ◽

pp. 1375-1377 ◽

Cited By ~ 5

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.

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

Canadian Journal of Gastroenterology ◽

10.1155/2000/423569 ◽

2000 ◽

Vol 14 (5) ◽

pp. 449-451 ◽

Cited By ~ 13

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.

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‘Prostate Abscess' as the Initial Manifestation of Granulomatosis with Polyangiitis (Wegener's Granulomatosis)

Urologia Internationalis ◽

10.1159/000363113 ◽

2014 ◽

Vol 96 (2) ◽

pp. 244-246 ◽

Cited By ~ 5

Author(s):

Sotirios Tsiodras ◽

Garyfalia Poulakou ◽

Konstantinos Leventakos ◽

Helen Panopoulou ◽

Antonia Elezoglou ◽

...

Keyword(s):

Wegener’S Granulomatosis ◽

Granulomatosis With Polyangiitis ◽

Systemic Disease ◽

Wegener's Granulomatosis ◽

Initial Manifestation ◽

Postoperative Fever ◽

Systemic Involvement ◽

Left Pleural Effusion ◽

Antibody Levels

Prostatic involvement in granulomatosis with polyangiitis (GWP), formerly known as Wegener's granulomatosis, is rare, mostly arising in the context of systemic involvement. Prostatic involvement as the first manifestation of this systemic disease is exceptionally rare. We hereby present the case of a 41-year-old male patient who underwent transurethral prostate resection for what was initially diagnosed as suppurative, focally necrotizing prostatitis. Prolonged postoperative fever that did not respond to various treatments, as well as the subsequent appearance of a left pleural effusion, a left upper pulmonary lobe lesion and cutaneous nodules, led to a reevaluation of histological slides which, along with the determination of serum c-ANCA/anti-PR3 antibody levels, established the diagnosis of GWP. Physicians, and especially urologists and infectious diseases specialists, should be aware of this rare association and consider GWP in the event of nonresolving prostatitis, especially when characteristic symptoms from other systems appear.

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