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

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

The Journal of Rheumatology ◽

10.3899/jrheum.100735 ◽

2011 ◽

Vol 38 (6) ◽

pp. 1048-1054 ◽

Cited By ~ 34

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.

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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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Prevention of relapses in Wegener's granulomatosis by treatment based on antineutrophil cytoplasmic antibody titre

The Lancet ◽

10.1016/0140-6736(90)92205-v ◽

1990 ◽

Vol 336 (8717) ◽

pp. 709-711 ◽

Cited By ~ 283

Author(s):

J COHENTERVAERT

Keyword(s):

Antibody Titre ◽

Wegener’S Granulomatosis ◽

Antineutrophil Cytoplasmic Antibody ◽

Wegener's Granulomatosis

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Myeloperoxidase-antineutrophil Cytoplasmic Antibodies with Cytoplasmic Fluorescence Pattern

Journal of Laboratory Physicians ◽

10.4103/0974-2727.66707 ◽

2010 ◽

Vol 2 (01) ◽

pp. 042-043 ◽

Cited By ~ 1

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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