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.

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.

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.

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 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 Wegener’s granulomatosis mimicking a parotid abscess

2005 ◽  
Vol 119 (9) ◽  
pp. 746-749 ◽  
Author(s):  
G L Jones ◽  
A D Lukaris ◽  
H V Prabhu ◽  
M J K M Brown ◽  
J Bondeson
Keyword(s):  
Salivary Gland ◽  
Parotid Gland ◽  
Wegener’S Granulomatosis ◽  
Wegener's Granulomatosis ◽  
Clinical Suspicion ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Surgical Exploration ◽  
First Case

We present the case of a previously healthy 59-year-old man who was under treatment for scleritis and episcleritis when he developed a parotid-gland swelling and pus-producing sinus. On surgical exploration, the features were those of a parotid abscess, but the lesion not only failed to heal post-operatively but increased in size very significantly. There was also severe necrotizing keratitis of the eyes. Due to clinical suspicion and a positive antineutrophil cytoplasmic antibodies test, Wegener’s granulomatosis was diagnosed and the patient successfully treated with cyclophosphamide and steroids. Previously, a number of cases of Wegener’s granulomatosis causing salivary-gland swelling have been reported in the literature; this is the first case in which the disease has masqueraded as a parotid abscess.

Wegener's Granulomatosis Presenting as Necrosis of the Left Mainstem Bronchus

2002 ◽  
Vol 10 (3) ◽  
pp. 277-279 ◽  
Author(s):  
Paul Schneider ◽  
Jörn Gröne ◽  
Jürgen Braun ◽  
Alejandra Perez-Canto ◽  
Heinz J Buhr
Keyword(s):  
Wegener’S Granulomatosis ◽  
Renal Involvement ◽  
Left Lung ◽  
Wegener's Granulomatosis ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Saddle Nose ◽  
Bronchial Arteries ◽  
Left Mainstem ◽  
Saddle Nose Deformity ◽  
Bronchial Inflammation

A patient with pansinusitis, nasal septum necrosis, and saddle nose deformity showed necrosis of the left mainstem, upper, and lower bronchi, with complete loss of left lung perfusion and ventilation. Pneumonectomy was performed. Histological findings showed extensive necrotizing and granulomatous bronchial inflammation with vasculitis of the bronchial arteries and the pulmonary vein. Wegener's granulomatosis was diagnosed, despite a negative cytoplasmic pattern of antineutrophil cytoplasmic antibodies and the lack of renal involvement.

scholarly journals Antineutrophil-cytoplasmic antibodies and antiglomerular basement membrane antibodies in Goodpasture's syndrome and in Wegener's granulomatosis.

1992 ◽  
Vol 2 (7) ◽  
pp. 1227-1234 ◽  
Author(s):  
M F Weber ◽  
K Andrassy ◽  
O Pullig ◽  
J Koderisch ◽  
K Netzer
Keyword(s):  
Basement Membrane ◽  
Wegener’S Granulomatosis ◽  
Wegener's Granulomatosis ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Globular Domain ◽  
Serum Samples ◽  
Double Positive ◽  
Clinical Prognosis ◽  
Histological Criteria ◽  
Circulating Autoantibodies

Antiglomerular basement membrane (anti-GBM) diseases-including Goodpasture's (GP) syndrome-and Wegener's granulomatosis (WG) are systemic diseases, which may be diagnosed by means of circulating autoantibodies. Possible overlap syndromes may exist; however, they remain imperfectly defined. We analyzed sera from 31 patients with WG and from 23 patients with anti-GBM disease. All underwent biopsy. Anti-cytoplasmic antibodies (ANCA) were demonstrated by indirect immunofluorescence (IIF); a perinuclear (P-ANCA) or diffuse-cytoplasmic (C-ANCA) staining was discerned. In addition, myeloperoxidase (MPO) antibodies (P-ANCA) and protein 3 (SP3) antibodies (C-ANCA) were analyzed by specific ELISA systems. Anti-GBM antibodies (anti-NC1 antibodies) were detected by ELISA and immunoblotting; the globular domain NC1 of collagen IV was employed as antigen. All 31 WG patients, as defined by clinical and histological criteria, showed ANCA by IIF. Twenty-nine of 31 showed a C-ANCA pattern; all were also positive for SP3 antibodies by ELISA. Three of 31 WG patients were P-ANCA positive by IIF and also had anti-MPO antibodies by ELISA. In one of these patients, SP3 antibodies were additionally found by IIF and by ELISA (double positive). No patient with WG had anti-NC1 antibodies. All 23 serum samples from patients with GP syndrome (N = 19) or anti-GBM glomerulonephritis (N = 4) had anti-NC1 antibodies. In seven of these patients, low titers of anti-MPO antibodies were detected by ELISA; however, the IIF for ANCA was negative. None of these seven patients had extraglomerular vasculities. In addition, the clinical prognosis of these patients was similar to that of those patients who lacked these antibodies.(ABSTRACT TRUNCATED AT 250 WORDS)

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