scholarly journals CLINICAL OBSERVATION OF THE PATIENT WITH WEGENER'S GRANULOMATOSIS

2017 ◽  
Vol 1 (66) ◽  
pp. 89-97
Author(s):  
Валерий Войцеховский ◽  
Valeriy Voytsekhovskiy ◽  
Марина Погребная ◽  
Marina Pogrebnaya ◽  
Николай Гоборов ◽  
...  
Keyword(s):  
Clinical Picture ◽  
Wegener’S Granulomatosis ◽  
Clinical Observation ◽  
Wegener's Granulomatosis ◽  
Tracheobronchial Tree ◽  
Acute Onset ◽  
Upper Respiratory Tract ◽  
Biopsy Material ◽  
Adequate Treatment ◽  
Upper Respiratory

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.

A Case of Generalized Wegener's Granulomatosis in Childhood: Successful Therapy With Cyclophosphamide

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
Keyword(s):  
Wegener’S Granulomatosis ◽  
Renal Involvement ◽  
Rare Condition ◽  
Wegener's Granulomatosis ◽  
Cytotoxic Agents ◽  
Upper Respiratory Tract ◽  
Pediatric Age Group ◽  
Upper Respiratory ◽  
Tract Infections

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.

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.

Combined Treatment in Wegener'S Granulomatosis with Crescentic Glomerulonephritis -Clinical Course and Longterm Outcome

1993 ◽  
Vol 16 (1) ◽  
pp. 11-19 ◽  
Author(s):  
G.M. Frascà ◽  
N.G. Zoumparidis ◽  
L.C. Borgnino ◽  
Lu. Neri ◽  
Lo. Neri ◽  
...  
Keyword(s):  
Serum Creatinine ◽  
Clinical Picture ◽  
Wegener’S Granulomatosis ◽  
Combined Treatment ◽  
Immunosuppressive Treatment ◽  
Clinical Signs ◽  
Wegener's Granulomatosis ◽  
Antithrombotic Agent ◽  
Methyl Prednisolone ◽  
Severe Clinical Picture

This study reports on 9 patients suffering from Wegener's granulomatosis (WG) with crescentic GN and severe systemic manifestations. On admission the mean serum creatinine was 10.9 ± 5.1 mg/dl (4-20 mg/dl); 8 patients were oliguric and required dialysis treatment. Renal biopsy showed crescents in all cases, involving 66 to 100% of glomeruli. Patients were treated with a protocol including: a plasmaexchange (PE) course; methyl-prednisolone; cyclophosphamide; and an antithrombotic agent (defibrotide). Clinical picture and renal function progressively improved in all patients within the first 4 weeks of treatment. After 1 month serum creatinine was 2.7 ± 0.8 mg/dl and dialysis was no longer needed in any patient. Five relapses occurred in 3 patients 12-26 months after the onset of the disease, while they were still receiving immunosuppressive treatment. At follow-up (22 to 112 months: mean 71) all patients were alive with no clinical signs of disease activity. One patient was on regular dialysis while the others had a serum creatinine of 1.2-2.8 mg/dl (mean 1.9). Our results confirm that crescentic GN associated with WG can be successfully treated even when associated with severe clinical picture and suggest that PE can contribute to control the disease without increasing immunosuppression.

Wegener's Granulomatosis Appearing Initially in the Trachea

1982 ◽  
Vol 91 (6) ◽  
pp. 593-594 ◽  
Author(s):  
J. Carlos Arauz ◽  
Rolando Fonseca
Keyword(s):  
Surgical Treatment ◽  
Clinical Picture ◽  
Wegener’S Granulomatosis ◽  
Open Surgery ◽  
Wegener's Granulomatosis ◽  
Tracheal Obstruction ◽  
Transient Improvement

We treated ten patients with Wegener's granulomatosis whose clinical picture differed only in the stage of the disease at the time of the first visit. All patients were female with a mean age of 30 years and all presented with a picture of high tracheal obstruction. Three died and six have a permanent tracheostomy. Surgical treatment was unsuccessful; corticosteroids provided only transient improvement but cyclophosphamide induced remission. Removal of the stenosis either by open surgery or endoscopically by laser was not successful in accomplishing decannulation.

Intranasal Mometasone Furoate for Treatment of Allergic Rhinitis

2010 ◽  
Vol 2 ◽  
pp. CMT.S4767 ◽  
Author(s):  
Marco Berlucchi ◽  
Barbara Pedruzzi
Keyword(s):  
Allergic Rhinitis ◽  
Clinical Picture ◽  
Mometasone Furoate ◽  
Upper Respiratory Tract ◽  
Nasal Symptoms ◽  
Pharmacodynamic Profile ◽  
Nasal Disease ◽  
Oral Steroids ◽  
Adults And Children ◽  
Upper Respiratory

Allergic rhinitis (AR) is a chronic nasal disease that affects the upper respiratory tract. This disorder is characterized by inflammation of the mucous membranes and it manifests with several nasal symptoms accompanied sometimes by non-nasal symptoms. Best therapy aims to prevent and improve the AR-clinical picture. Steroids have an important role in the treatment of AR. The development of steroids administrated directly on nasal mucosa has much reduced the systemic adverse affects associated with oral steroids therapy. Mometasone furoate aqueous nasal spray is a synthetic steroid assessed for intranasal use in the therapy of adults and children affected by AR. Such topical nasal steroid is an effective molecule improving clinical picture of AR and it is also approved as prophylactic therapy. In this article, apart from a careful description of its successful clinical use the authors review pharmacokinetic/pharmacodynamic profile, mechanism of action, safety, and efficacy of such steroid molecule.

scholarly journals Difficulties in the Differential Diagnosis of Tracheobronchomegaly in Children

2020 ◽  
Vol 101 (3) ◽  
pp. 175-182
Author(s):  
N. A. Il’ina ◽  
L. E. Vorob’eva ◽  
M. D. Bakradze ◽  
A. S. Polyakova
Keyword(s):  
Cystic Fibrosis ◽  
Connective Tissue ◽  
Pulmonary Fibrosis ◽  
Connective Tissue Diseases ◽  
Clinical Manifestations ◽  
Tracheobronchial Tree ◽  
Developmental Defect ◽  
Upper Respiratory Tract ◽  
Upper Respiratory

Tracheobronchomegaly is a rare developmental defect in children, which is characterized by the expansion of the trachea and large bronchi, the diagnosis and treatment of which are made more often in adult practice, when the clinical diagnostic picture acquires the obvious features of Mounier-Kuhn syndrome. In this connection, the need arises for the early detection of this pathology in children and its differentiation from other diseases and malformations of the tracheobronchial tree, which mask a recurrent inflammatory process in the lung and upper respiratory tract, and primarily with those, such as cystic fibrosis and primary ciliary dyskinesia.The paper describes a case of secondary tracheobronchomegaly in a child with obvious clinical manifestations of Mounier-Kuhn syndrome in the presence of bronchiectasis, pulmonary fibrosis, and signs of systemic connective tissue disease. It discusses the possibilities of chest computed tomography in the diagnosis of this defect in children, as well as its causes. The sizes of the trachea and main bronchi in this patient are compared with those in the groups of children of the same age and sex who have cystic fibrosis or diffuse pulmonary fibrosis to identify a more significant role of hereditary connective tissue diseases in the development of secondary tracheobronchomegaly. The authors propose to separate the concepts of tracheobronchomegaly and Mounier-Kuhn syndrome in children and to recognize the threshold for tracheal expansion in these patients having +3SD or more.

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