Wegener's Granulomatosis, Systemic Vasculitis, and Antineutrophil Cytoplasmic Autoantibodies

1991 ◽  
Vol 42 (1) ◽  
pp. 459-469 ◽  
Author(s):  
R J Falk ◽  
J C Jennette
Keyword(s):  
Wegener’S Granulomatosis ◽  
Systemic Vasculitis ◽  
Wegener's Granulomatosis ◽  
Antineutrophil Cytoplasmic Autoantibodies

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 and Antineutrophil Cytoplasmic Autoantibodies

1993 ◽  
Vol 102 (11) ◽  
pp. 906-908 ◽  
Author(s):  
John G. Batsakis ◽  
Adel K. El-Naggar
Keyword(s):  
Laboratory Test ◽  
Wegener’S Granulomatosis ◽  
Wegener's Granulomatosis ◽  
Proteinase 3 ◽  
Inflammatory Disorders ◽  
Serologic Tests ◽  
Criteria For Diagnosis ◽  
Antineutrophil Cytoplasmic Autoantibodies

Serologic tests for antineutrophil cytoplasmic autoantibodies can serve as markers for a number of necrotizing vasculitides and other inflammatory disorders. In the case of Wegener's granulomatosis, an immunofluorescent cytoplasmic pattern and immunoassay for proteinase 3 are quite specific for the disease and are capable of serving as additional criteria for diagnosis. As with any laboratory test, however, results are to be interpreted in the whole clinical and pathologic framework 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 Evaluation of 10-Year Experience of Wegener’s Granulomatosis in Iranian Children

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Fatemeh Tahghighi ◽  
Mohamad-Hassan Moradinejad ◽  
Yahya Aghighi ◽  
Reza Shiari ◽  
Seyed-Reza Raeeskarami ◽  
...  
Keyword(s):  
Wegener’S Granulomatosis ◽  
Pediatric Patients ◽  
Systemic Vasculitis ◽  
Wegener's Granulomatosis ◽  
Organ System ◽  
Average Delay ◽  
Tract Involvement ◽  
Eye Involvement ◽  
The Mean ◽  
Iranian Children

Wegener’s granulomatosis or granulomatosis polyangiitis (GPA) is an uncommon chronic systemic vasculitis in children. The aim of this study was to describe pediatric patients with GPA in Iran. We studied records of all patients with GPA diagnosis who were referred to all Iranian Pediatric Rheumatology Division from 2002 to 2011. A total of 11 patients (5 females and 6 males) enrolled in this study. In children less than 15 years old, the prevalence of GPA is 0.6 per million. The mean age of GPA diagnosis was 11 years and average delay diagnosis was 20 months. Mortality rate was 18.1% due to pulmonary vasculitis and infection. The most common organ system involvement was upper and lower respiratory tract involvement (81.8% and 63.9%, resp.). Other common manifestations were renal (36.3), skin (27.2%), and eye involvement (18.2%).

Negative ANCA in Wegener's granulomatosis

1994 ◽  
Vol 108 (5) ◽  
pp. 420-422 ◽  
Author(s):  
S. Carrie ◽  
K. B. Hughes ◽  
M. G. Watson
Keyword(s):  
Early Diagnosis ◽  
Poor Prognosis ◽  
Wegener’S Granulomatosis ◽  
Systemic Vasculitis ◽  
Wegener's Granulomatosis ◽  
Clinical Suspicion ◽  
Diagnosis And Treatment ◽  
Serological Marker ◽  
Antineutrophil Cytoplasmic Antibodies

AbstractWegener's granulomatosis is a systemic vasculitis characterized by necrotizing granulomata and without treatment is associated with a poor prognosis. Antineutrophil cytoplasmic antibodies (ANCA) have proved to be a useful serological marker allowing for early diagnosis and treatment of this condition. We report two cases of patients who were ANCA-negative on presentation despite clinical suspicion of Wegener's granulomatosis. In both cases the patients developed a positive ANCA titre as the disease progressed.

scholarly journals HLA antigen frequencies in systemic vasculitis: increase in HLA-DR2 in wegener's granulomatosis

1983 ◽  
Vol 26 (1) ◽  
pp. 102-105 ◽  
Author(s):  
Keith B. Elkon ◽  
David C. Sutherland ◽  
Andrew J. Rees ◽  
Graham R. V. Hughes ◽  
J. Richard Batchelor
Keyword(s):  
Wegener’S Granulomatosis ◽  
Systemic Vasculitis ◽  
Wegener's Granulomatosis ◽  
Hla Antigen

scholarly journals Orbital presentation of systemic vasculitis: a diagnostic and management challenge

2015 ◽  
Vol 7 (1) ◽  
pp. 65-68 ◽  
Author(s):  
M Singh ◽  
U Singh ◽  
Z Zadeng
Keyword(s):  
Wegener’S Granulomatosis ◽  
Vision Loss ◽  
Systemic Vasculitis ◽  
Wegener's Granulomatosis ◽  
Good Prognosis ◽  
Saddle Nose ◽  
Orbital Mass ◽  
Saddle Nose Deformity ◽  
Ulcerative Lesions ◽  
Management Challenge

Background: Orbital involvement in Wegener’s Granulomatosis (WG) is rare and has an overall good prognosis. Case: A 60-year-old hypertensive Indian female presented with vision loss and painful proptosis of left eye. Orbital incisional biopsy suggested necrotising small vessel inflammation. The saddle nose deformity and pedal nodulo- ulcerative lesions further consolidated the diagnosis of Wegener’s granulomatosis. Systemic immunosuppressant provided remission and the only relapse was managed successfully with intravenous Rituximab. Conclusion: Wegener’s granulomatosis should be kept in the differential diagnosis of painful proptosis with a diffuse orbital mass in an elderly patient presenting with profound vision loss. Newer immune modulating agents are useful adjuncts in preventing relapses of this fatal disease.

scholarly journals A Diagnosis Missed for Several Years- Wegener’s Granulomatosis

2012 ◽  
Vol 9 (3) ◽  
pp. 218-221 ◽  
Author(s):  
B P Paudyal ◽  
S Pantha ◽  
N Ranjitkar ◽  
A Manandhar ◽  
A Arjyal
Keyword(s):  
Wegener’S Granulomatosis ◽  
Systemic Vasculitis ◽  
Systemic Disease ◽  
Granulomatous Inflammation ◽  
Wegener's Granulomatosis ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Upper Respiratory Tract ◽  
Delay In Diagnosis ◽  
Organ Systems ◽  
Wide Range

Wegener’s granulomatosis is a form of systemic vasculitis of small to medium sized vessels and affects upper respiratory tract, lungs and kidneys along with various organs. It causes necrotizing granulomatous inflammation of the affected parts and presents with positive antineutrophil cytoplasmic antibodies in more severe forms. Being a systemic disease with the potential to affect any organ-systems with a wide range of clinical presentations, it is associated with a risk of delay in diagnosis with resultant setback in institution of appropriate treatment. Confusion may arise due to an extent of histological similarity between Wegener’s granulomatosis and the more prevalent tuberculosis, both causing granulomatous inflammation of the affected parts. Here, we present two cases of this rare disorder where the diagnosis was missed for several years in the beginning causing a delay in institution of specific therapy which led to the development of complications.DOI: http://dx.doi.org/10.3126/kumj.v9i3.6309 Kathmandu Univ Med J 2011;9(3):218-21 

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