scholarly journals Toxic epidermal necrolysis and concurrent granulomatosis with polyangiitis (Wegener’s granulomatosis). Management of a rare case and review of the literature

2016 ◽  
Vol 2 ◽  
pp. 205951311664212
Author(s):  
Stratos S Sofos ◽  
J Ewing ◽  
LC Hughes ◽  
MI James
Keyword(s):  
Toxic Epidermal Necrolysis ◽  
Wegener’S Granulomatosis ◽  
Rare Case ◽  
Granulomatosis With Polyangiitis ◽  
Wegener's Granulomatosis ◽  
Stevens Johnson Syndrome ◽  
Review Of The Literature ◽  
Systemic Corticosteroids ◽  
Burn Unit ◽  
Necrotising Vasculitis

[Formula: see text] Toxic epidermal necrolysis (TEN) is a rare, acute life-threatening mucocutaneous disorder that is characterised by epidermal loss/exfoliation exceeding 30% total body surface area (TBSA) and is on a spectrum that includes erythema multiforme and Stevens–Johnson syndrome (SJS). It is estimated that 80% of TEN cases are related to medication reactions; the association based on the recognition that TEN usually develops 1–3 weeks following administration of the suspect drug. It is agreed that primary treatment consists of prompt withdrawal of causative drugs and transfer to a regional burn unit. Transfer to a burn unit, no more than 7 days after onset of symptoms, has been acknowledged as reducing the risk of infections, hospital length of stay and infection-related mortality. Due to the uncertainty surrounding TEN pathogenesis, several different modalities have been proposed for the treatment of TEN, including high-dose intravenous immunoglobulins, plasmapheresis, cyclophosphamide, cyclosporine and systemic steroids; however, these therapies are relatively ineffective. The use of systemic corticosteroids for treatment of TEN has in particular been deemed controversial due to associations with increased infections leading to greater length of hospital stay and increased mortality. Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a rare relapsing-remitting disorder of unknown aetiology, characterised by granulomatosis inflammation and necrotising vasculitis predominantly affecting small- to medium-sized vessels. While a 5-year survival rate of 75–83% is now realised, relapse and associated morbidity is of concern. The established treatment for GPA follows the recommendations of the French National Authority for Health (HAS) for systematic necrotising vasculitis. With induction treatment, it is recommended that GPA be treated with a combination of systemic corticosteroids and immunosuppressants. A review of the literature failed to identify any previous case where both of these conditions coincide. Our search was conducted through databases which included MEDLINE, PubMed, Scopus, AMED, CINAHL and EMBASE, using keywords: toxic epidermal necrolysis, Wegener’s granulomatosis, granulomatosis with polyangiitis. We submit the rare case of a 22-year-old woman who presented to our regional burn unit with both GPA and TEN, and we discuss the presentation, investigation and multidisciplinary management of the patient, as well as reviewing the literature regarding these two conditions.

scholarly journals A Case of Granulomatosis with Polyangiitis (Wegener’s Granulomatosis) Presenting with Marked Inflamed Tracheobronchial Mucosa

2013 ◽  
Vol 2013 ◽  
pp. 1-5
Author(s):  
Teruaki Nishiuma ◽  
Hisashi Ohnishi ◽  
Sho Yoshimura ◽  
Saori Kinami ◽  
Susumu Sakamoto
Keyword(s):  
Weight Loss ◽  
Wegener’S Granulomatosis ◽  
Rare Case ◽  
Granulomatosis With Polyangiitis ◽  
Giant Cells ◽  
Wegener's Granulomatosis ◽  
Transbronchial Biopsy ◽  
Multinucleated Giant Cells ◽  
Diffuse Erythema ◽  
Localized Form

A 70-year-old man was admitted to our hospital because of weight loss and persistent dry cough. Chest radiograph and CT showed multiple infiltrates in the bilateral upper lobes and the remarkably thickened bronchial walls. Bronchoscopy revealed diffuse erythema and edema of the tracheobronchial mucosa without any ulcerous legions. Serum MPO-ANCA was positive (155 EU). Transbronchial biopsy was performed and revealed necrotic granulomas with multinucleated giant cells in the bronchial/bronchiolar and parenchymal lesions. Thus, we diagnosed it as a localized form of granulomatosis with polyangiitis (GPA, Wegener’s granulomatosis). After treatment with corticosteroid and cyclophosphamide, the bronchial findings were entirely resolved. We report here a rare case of GPA presenting with markedly inflamed tracheobronchial mucosa.

scholarly journals Bilateral Ureteral Stenosis with Hydronephrosis as First Manifestation of Granulomatosis with Polyangiitis (Wegener’s Granulomatosis): A Case Report and Review of the Literature

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Joelle Suillot ◽  
Jürg Bollmann ◽  
Samuel Rotman ◽  
Eric Descombes
Keyword(s):  
Wegener’S Granulomatosis ◽  
Crescentic Glomerulonephritis ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Wegener's Granulomatosis ◽  
High Dose ◽  
Review Of The Literature ◽  
Ureteral Stenosis ◽  
Rare Manifestation ◽  
Unilateral Hydronephrosis

Ureteral stenosis is a rare manifestation of granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis). We report the case of a 76-year-old woman with progressive renal failure in which bilateral hydronephrosis due to ureteral stenosis was the first manifestation of the disease. Our patient also had renal involvement with pauci-immune crescentic glomerulonephritis associated with high titers of anti-proteinase 3 c-ANCAs, but no involvement of the upper or lower respiratory tract. The hydronephrosis and renal function rapidly improved under immunosuppressive therapy with high-dose corticosteroids and intravenous pulse cyclophosphamide. We reviewed the literature and found only ten other reported cases of granulomatosis with polyangiitis/Wegener’s granulomatosis and intrinsic ureteral stenosis: in two cases, the presenting clinical manifestation was unilateral hydronephrosis and in only two others was the hydronephrosis bilateral, but this complication developed during a relapse of the disease. This case emphasizes the importance of including ANCA-related vasculitis in the differential diagnosis of unusual cases of unilateral or bilateral ureteral stenosis.

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

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

Sign in / Sign up

Export Citation Format

Share Document