Granulomatosis with Polyangiitis (Wegener's Granulomatosis): Evolving Concepts in Treatment

2018 ◽  
Vol 39 (04) ◽  
pp. 434-458 ◽  
Author(s):  
Ariis Derhovanessian ◽  
Henry Tazelaar ◽  
John Belperio ◽  
Joseph Lynch
Keyword(s):  
Respiratory Tract ◽  
Wegener’S Granulomatosis ◽  
Granulomatosis With Polyangiitis ◽  
Treatment Strategies ◽  
Granulomatous Inflammation ◽  
Wegener's Granulomatosis ◽  
Upper Respiratory Tract ◽  
Small Vessels ◽  
Successful Induction ◽  
Indolent Disease

AbstractGranulomatosis with polyangiitis (GPA), formerly termed Wegener's granulomatosis, is the most common of the pulmonary vasculitides. GPA typically involves the upper respiratory tract, lower respiratory tract (bronchi and lung), and kidney, with varying degrees of disseminated vasculitis. Cardinal histologic features include a necrotizing vasculitis involving small vessels, extensive “geographic” necrosis, and granulomatous inflammation. The spectrum and severity of the disease is heterogeneous, ranging from indolent disease involving only one site to fulminant, multiorgan vasculitis. Circulating antibodies against cytoplasmic components of neutrophils (ANCAs) play a role in the pathogenesis, and often correlate with activity of the disease. Treatment strategies are evolving. Cyclophosphamide (CYC) plus corticosteroids was the mainstay of therapy for generalized, multisystemic GPA since the 1970s. However, within the past decade, rituximab (RTX), a monoclonal antibody directed against B cells, has been shown to be at least as effective (and possibly more effective) as CYC. Furthermore, the use of RTX may reduce the need for maintenance immunosuppression. Optimal therapy for GPA remains controversial, and additional studies are required to determine the role and duration of maintenance therapy following successful induction therapy.

scholarly journals GRANULOMATOSIS WITH POLYANGIITIS: TREAT THE PATIENT NOT SYMPTOMS

2018 ◽  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Treatment Strategies ◽  
Granulomatous Inflammation ◽  
Organ Damage ◽  
Upper Respiratory Tract ◽  
Childbearing Age ◽  
Immunological Mechanisms ◽  
Upper Respiratory ◽  
Tract Infections ◽  
In Pregnancy

Immunological mechanisms of appearance and therapeutic treatment strategies were discussed on example of the rare granulomatosis with polyangiitis clinical case in young patient. This vasculitis, formerly known as Wegener’s granulomatosis, is a rare multisystem autoimmune disease with necrotizing granulomatous inflammation and pauci-immune vasculitis in small- and medium-sized blood vessels. Autoimmune diseases affect 5 to 7% of people, are commoner in women of childbearing age, and are frequently encountered in pregnancy. They may remit or improve during pregnancy, butcan flare or present in pregnancy with disastrous consequences. Otorhinolaryngologist is the first physician to contact for the majority of patients with GPA. This diagnosis must always be taken into consideration in patients with recurrent upper respiratory tract infections, otitis, mucosal ulcers and laryngitis. Proper and early diagnosis is crucial for imminent therapy implementation and allows avoiding irreversible organ damage.

scholarly journals Evolution of sinonasal radiological findings in a case of granulomatosis with polyangiitis

2019 ◽  
Vol 9 (33) ◽  
pp. 43-48
Author(s):  
Aru Chhabra Handa ◽  
Anup Singh ◽  
Jai Prakash Sharma
Keyword(s):  
Wegener’S Granulomatosis ◽  
Granulomatosis With Polyangiitis ◽  
Bone Erosion ◽  
Granulomatous Inflammation ◽  
Wegener's Granulomatosis ◽  
Unknown Etiology ◽  
Saddle Nose ◽  
Radiological Findings ◽  
Radiological Abnormality ◽  
Granulomatous Disorder

Abstract Wegener’s granulomatosis (Granulomatosis with Polyangiitis) is an autoimmune granulomatous disorder of unknown etiology. The basic pathology is necrotizing granulomatous inflammation with necrotizing vasculitis involving medium-sized blood vessels. The typical triad of involvement of the upper and lower respiratory tracts and the kidneys is observed in varying proportions in individual patients. The sinonasal involvement is observed in the majority of patients and it can be involved in isolation in up to a quarter of affected individuals. The sinonasal changes involve both the soft tissue and bony architecture, with refractory chronic sinusitis associated with erosion of the cartilaginous and bony skeleton and may lead to saddle nose and other facial deformities in advanced cases. The radiology has a contributory role as diagnostic and diseases monitoring aid. The various radiological findings associated with the disease (bone erosion with osteoneogenesis) have been mentioned in the literature. We present the progression of a radiological abnormality in a patient with a localized form of Wegener’s Granulomatosis and highlight some peculiar appearances observed on radiology in the patient.

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 

Tuberculosis of the upper respiratory tract misdiagnosed as Wegener's granulomatosis—an important distinction

1990 ◽  
Vol 104 (3) ◽  
pp. 255-258 ◽  
Author(s):  
A. D. Couldery
Keyword(s):  
Respiratory Tract ◽  
Wegener’S Granulomatosis ◽  
Wegener's Granulomatosis ◽  
Upper Respiratory Tract ◽  
Bacteriological Examination ◽  
Important Distinction ◽  
Upper Respiratory

AbstractFour cases, two personal, of mis-diagnosis of tuberculosis of the upper respiratory tract as Wegener's granulomatosis have been presented. Greater awareness of this possibility of mis-diagnosis may diminish the possibility of mistreatment. It is suggested that fresh specimens should be sent for bacteriological examination and culture in all relevant upper respiratory tract lesions.

scholarly journals Panhypopituitarism due to Wegener's granulomatosis

2011 ◽  
Vol 55 (7) ◽  
pp. 481-485 ◽  
Author(s):  
Silvina G. Santoro ◽  
Alberto H. Guida ◽  
Alejandra E. Furioso ◽  
Patricia Glikman ◽  
Amelia S. Rogozinski
Keyword(s):  
Literature Review ◽  
Respiratory Tract ◽  
Diabetes Insipidus ◽  
Endocrine Therapy ◽  
Wegener’S Granulomatosis ◽  
Wegener's Granulomatosis ◽  
Upper Respiratory Tract ◽  
Granulomatous Vasculitis ◽  
Pituitary Enlargement ◽  
Upper Respiratory

Wegener's granulomatosis (WG) is a multi-system necrotizing granulomatous vasculitis which classically affects the upper respiratory tract, lungs and kidneys. Pituitary participation has been described in 24 patients in the literature to date. The aim of this article is to report a case of pituitary involvement in WG, and to present a literature review on this association. We present a female patient with WG who evolved with central diabetes insipidus (CDI), panhypopituitarism, and mild hyperprolactinemia. MRI showed an infiltrative pattern. Pituitary involvement has been reported in around 1% of patients with WG, mostly in women. It is represented by CDI and hypopituitarism. MRI generally shows pituitary enlargement, stalk thickening and loss of hyperintensity of the neurohypophysis. Permanent endocrine therapy is generally needed. WG should be considered in cases of CDI and hypopituitarism, essentially if a vasculitis is suspected and more common sellar disorders have been ruled out.

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 Severe course of quickly progressing glomerulonephritis in a patient with ANTSA-associated vasculitis: review and case report

2020 ◽  
Vol 7 (3) ◽  
pp. 55-62
Author(s):  
Iu. V. Lavrishcheva ◽  
Y. S. Kaledinova ◽  
A. A. Yakovenko ◽  
I. A. Artemev
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Rapidly Progressive Glomerulonephritis ◽  
Granulomatous Inflammation ◽  
Clinical Work ◽  
Chronic Hemodialysis ◽  
Complete Loss ◽  
Vascular Lesions ◽  
Upper Respiratory Tract ◽  
Delay In Diagnosis ◽  
Necrotizing Glomerulonephritis

Granulomatosis with polyangiitis is characterized by necrotizing granulomatous inflammation, vasculitis with vascular lesions of small and medium caliber and focal necrotizing glomerulonephritis. A frequent and one of the most formidable complications is kidney damage, which in a large number of cases leads to a complete loss of organ function and a switch to renal replacement therapy. Given the rare occurrence of this disease in the clinical work of practitioners, and their low awareness of this pathology, problems often arise with the diagnosis and treatment of patients with HPA. Due to the diversity and non-specific nature of the manifestations of the disease, a delay in diagnosis may occur. The presented case illustrates the manifestations of granulomatosis with polyangiitis in the form of severe damage to the upper respiratory tract and kidneys, the diagnosis of which was difficult due to the rarity of the disease and the multiple organ pathology. This article presents a clinical case of severe progression of rapidly progressive glomerulonephritis in a patient with ANCA-associated vasculitis, a brief review of the literature is given. Despite adequate therapy, the disease progressed mainly due to deterioration of renal function, which subsequently led to a complete loss of kidney function and the transition to treatment with chronic hemodialysis.

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