Wegener's Granulomatosis Appearing Initially in the Trachea

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.

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

The International Journal of Artificial Organs ◽

10.1177/039139889301600104 ◽

1993 ◽

Vol 16 (1) ◽

pp. 11-19 ◽

Cited By ~ 11

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.

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CLINICAL OBSERVATION OF THE PATIENT WITH WEGENER'S GRANULOMATOSIS

Bulletin physiology and pathology of respiration ◽

10.12737/article_5a24d1a5e1f301.09080009 ◽

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.

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Successful Surgical Treatment of Massive Lower Gastrointestinal Bleeding in a Patient with Wegener's Granulomatosis

The American Surgeon ◽

10.1177/000313481207800914 ◽

2012 ◽

Vol 78 (9) ◽

pp. 416-417

Author(s):

Gülay Koçak ◽

Erdem Koçak ◽

Bülent Huddam ◽

Alper Azak ◽

Nuray Voyvoda ◽

...

Keyword(s):

Surgical Treatment ◽

Gastrointestinal Bleeding ◽

Wegener’S Granulomatosis ◽

Wegener's Granulomatosis ◽

Lower Gastrointestinal Bleeding

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Wegener's Granulomatosis of the Subglottis and the Upper Portion of the Trachea

Annals of Otology Rhinology & Laryngology ◽

10.1177/000348948209100610 ◽

1982 ◽

Vol 91 (6) ◽

pp. 588-592 ◽

Cited By ~ 57

Author(s):

Thomas J. McDonald ◽

H. Bryan Neel ◽

Richard A. DeRemee

Keyword(s):

Medical Treatment ◽

Reconstructive Surgery ◽

Paranasal Sinuses ◽

Wegener’S Granulomatosis ◽

Wegener's Granulomatosis ◽

Tracheotomy Tube ◽

Tracheal Obstruction ◽

Airway Symptoms ◽

Nose And Paranasal Sinuses

A relatively little-known complication of Wegener's granulomatosis is involvement of the subglottis and upper part of the trachea, which is illustrated in this report on 17 patients. Each of the patients had biopsy-proved Wegener's granulomatosis of the nose and paranasal sinuses. Some had concomitant involvement of the eye, kidney, lung and skin. Nine of the 17 patients required tracheotomy; 5 still require a tracheotomy tube, and 4 have had decannulation (1 had successful reconstructive surgery of the trachea). Medical treatment consisted of cyclophosphamide, with or without corticosteroids. All 17 are currently alive, the average follow-up having been 81 months. The otolaryngologist plays a key role in the detection and management of patients with Wegener's granulomatosis, particularly when the onset is a sudden progressive upper tracheal obstruction with airway symptoms that may overshadow the sometimes more subtle nasal manifestations.

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