Ear, Nose and Throat Manifestations of Wegener's Granulomatosis (Granulomatosis With Polyangiitis)

Granulomatosis with polyangiitis (Wegener’s granulomatosis) is one of the idiopathicimmune-mediated small-vessel vasculitides described in humans which are characterised bythe presence of circulating antineutrophil cytoplasmic antibodies. It most commonly involvescapillaries, venules and arterioles of the ear, nose and throat, lungs and glomeruli. A caseof destructive haemopurulent rhinitis associated with relapsing periods of pyrexia, lethargyand stiffness as well as generalised pulmonary infiltrates in a young Anatolian shepherddog is presented that closely resembles granulomatosis with polyangiitis (GPA) as reportedin humans. Perinuclear antineutrophil cytoplasmic antibodies (pANCA) were detectedin the dog’s serum. Signs resolved promptly and completely once immunosuppressivedoses of prednisone were administered, and have not recurred. This is the first report onthe use of pANCA to investigate rhinitis in dogs. It is also, to the authors’ knowledge,the first description of a relapsing haemopurulent lytic rhinitis in this species. Theconcurrent manifestations of erosive haemopurulent rhinitis, ground-glass opacities onpulmonary computed tomography, pyrexia and listlessness resemble GPA as described inhumans.

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Clinical manifestations of granulomatosis with polyangiitis (Wegener’s granulomatosis) in the upper respiratory tract seen by otolaryngologists in Japan

Clinical and Experimental Nephrology ◽

10.1007/s10157-012-0768-2 ◽

2013 ◽

Vol 17 (5) ◽

pp. 663-666 ◽

Cited By ~ 12

Author(s):

Yasuaki Harabuchi ◽

Kan Kishibe ◽

Yuki Komabayashi

Keyword(s):

Respiratory Tract ◽

Wegener’S Granulomatosis ◽

Granulomatosis With Polyangiitis ◽

Clinical Manifestations ◽

Wegener's Granulomatosis ◽

Upper Respiratory Tract ◽

Upper Respiratory

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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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57 Granulomatosis with Polyangiitis (Wegener’s Granulomatosis)

Otolaryngology Cases ◽

10.1055/b-0038-160743 ◽

2018 ◽

Keyword(s):

Wegener’S Granulomatosis ◽

Granulomatosis With Polyangiitis ◽

Wegener's Granulomatosis

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Granulomatosis with Polyangiitis-GPA (Wegener’s Granulomatosis)

10.1007/978-981-16-4047-6_1 ◽

2021 ◽

pp. 3-12

Author(s):

Sanjana Vijay Nemade ◽

Kiran Jaywant Shinde

Keyword(s):

Wegener’S Granulomatosis ◽

Granulomatosis With Polyangiitis ◽

Wegener's Granulomatosis

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Rheumatoid Arthritis and Granulomatosis With Polyangiitis (Wegener’s Granulomatosis): A Rare Association

Bangladesh Journal of Medicine ◽

10.3329/bjm.v31i2.48542 ◽

2020 ◽

Vol 31 (2) ◽

pp. 114-119

Author(s):

Wafia Najifa ◽

Mohiuddin Sharif ◽

Rajib Roy ◽

Mahfuzul Haque ◽

Md Robed Amin

Keyword(s):

Rheumatoid Arthritis ◽

Clinical Practice ◽

Wegener’S Granulomatosis ◽

Granulomatosis With Polyangiitis ◽

Wegener's Granulomatosis ◽

Overlap Syndrome ◽

Wegener Granulomatosis ◽

Overlap Syndromes ◽

Rare Association

The presentation of Rheumatoid arthritis (RA) combined with a second rheumatological disorder thatis, different RA overlap syndromes are frequently encountered in clinical practice. But RA-vasculitisoverlaps are relatively rare. This paper presents a case of Rheumatoid arthritis and Granulomatosiswith polyangiitis (Wegener granulomatosis) overlap syndrome which is first of its kind reported from Bangladesh. Bangladesh J Medicine July 2020; 31(2) : 114-119

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Granulomatosis (Wegener's granulomatosis) with polyangiitis presented as pulmonary manifestation: a case report

Respirology Case Reports ◽

10.1002/rcr2.674 ◽

2020 ◽

Vol 8 (8) ◽

Author(s):

Qusay Jummaa Lazim ◽

Sinan Shakir Gheni Atrah ◽

Khalid Jawad Mutlag ◽

Haider Saadoon Qasim Alhilfi ◽

Ahmed Muhi Fahad ◽

...

Keyword(s):

Case Report ◽

Wegener’S Granulomatosis ◽

Granulomatosis With Polyangiitis ◽

Wegener's Granulomatosis ◽

Pulmonary Manifestation

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Ear, nose, and throat symptoms in subacute Wegener's granulomatosis.

BMJ ◽

10.1136/bmj.299.6702.791 ◽

1989 ◽

Vol 299 (6702) ◽

pp. 791-791

Keyword(s):

Wegener’S Granulomatosis ◽

Wegener's Granulomatosis ◽

Nose And Throat

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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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