Wegener's granulomatosis upper respiratory tract and pulmonary radiographic manifestations in 30 cases with pathogenetic consideration

1998 ◽  
Vol 22 (2) ◽  
pp. 99-104 ◽  
Author(s):  
Myung S Shin ◽  
K.Randall Young ◽  
Kang-Jey Ho

1990 ◽  
Vol 104 (3) ◽  
pp. 255-258 ◽  
Author(s):  
A. D. Couldery

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.



2011 ◽  
Vol 55 (7) ◽  
pp. 481-485 ◽  
Author(s):  
Silvina G. Santoro ◽  
Alberto H. Guida ◽  
Alejandra E. Furioso ◽  
Patricia Glikman ◽  
Amelia S. Rogozinski

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.



Author(s):  
Валерий Войцеховский ◽  
Valeriy Voytsekhovskiy ◽  
Марина Погребная ◽  
Marina Pogrebnaya ◽  
Николай Гоборов ◽  
...  

A brief review of the literature is devoted to the peculiarities of the clinical picture, diagnosis and treatment of Wegener's granulomatosis. The clinical observation of the patient with Wegener's granulomatosis is made from the personal practice of the authors. A complex differential diagnosis was made between pneumonia of different etiology, tuberculosis, lung cancer and granulomatous disease. Taking into account anamnesis and clinical picture of the disease (acute onset with fever, arthralgia, nasal and oral mucosa damage, lungs, kidneys, Raynaud's syndrome, hemorrhagic eruptions, digital vasculitis), histological examination of transbronchial lung tissue biopsy material diagnosed: Wegener's granulomatosis, a generalized form, with the lesions of the upper respiratory tract, tracheobronchial tree, lungs, heart, kidneys, an acute course. Despite adequate treatment, the disease progressed with the addition of various complications, and a lethal outcome was ascertained.



2018 ◽  
Vol 39 (04) ◽  
pp. 434-458 ◽  
Author(s):  
Ariis Derhovanessian ◽  
Henry Tazelaar ◽  
John Belperio ◽  
Joseph Lynch

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.



PEDIATRICS ◽  
1978 ◽  
Vol 61 (2) ◽  
pp. 286-290
Author(s):  
Radhakrishna Baliga ◽  
Chung-Ho Chang ◽  
Anil K. Bidani ◽  
Eugene V. D. Perrin ◽  
Larry E. Fleischmann

An 11-year-old white boy had Wegener's granulomatosis, a rare condition in the pediatric age group. The clinical course, pathological findings, and mode of treatment are outlined. The disease is in remission on a regimen of cyclophosphamide therapy as judged by both clinical and pathological criteria. This syndrome with protean manifestations should be considered in children with symptoms of repeated upper respiratory tract infections along with pulmonary and renal involvement. Early renal biopsy helps to establish the diagnosis of generalized involvement and to guide the course of treatment. Follow-up renal biopsies may serve as an indication for the continuation of treatment. Cytotoxic agents, especially cyclophosphamide, dramatically alter the course of the disease.



1970 ◽  
Vol 3 (2) ◽  
pp. 265-276 ◽  
Author(s):  
Jack D. Clemis ◽  
Eugene L. Derlacki


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