Radiation Therapy and Mechanical Dilation of Endobronchial Obstruction Secondary to Wegener's Granulomatosis

Eagleton, Lanie E.; Rosher, Richard B.; Hawe, Anthony; Bilinsky, Richard T.

doi:10.1378/chest.76.5.609

Cited by 19 publications

(10 citation statements)

References 4 publications

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“…Cyclophosphamide concomitantly with prednisone is the treatment of choice as the first line procedure [2–4, 8, 11, 12, 14–17]. In the remission phase or in patients with stable disease, azathioprine, methotrexate and glucocorticoids are routinely used.…”

Section: Discussionmentioning

confidence: 99%

“…Very limited data concerning radiation therapy results have been published in the literature so far, but case reports showed a significant response rate [11, 13]. The routinely used radiation dose ranges between 40 and 50 Gy with conventional fractionation.…”

Section: Discussionmentioning

confidence: 99%

“…Radiotherapy may be used in selected patients with Wegener's granulomatosis refractory to standard treatment [11–13]. It is possible to perform it locally, except for progressive disease, even when it concerns critical organs or is located in their close proximity.…”

Section: Introductionmentioning

confidence: 99%

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Low dose radiotherapy as an effective treatment in a patient with solitary Wegener’s granulomatosis resistant to systemic treatment –

Wygoda¹,

Rutkowski²,

Składowski³

et al. 2013

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Wegener's granulomatosis is a rare disease (10 per 1 000 000 new cases per year), etiologically connected with necrotizing vasculitis of small to medium-size vessels. The disease occurs predominantly in the upper respiratory tract, lungs and kidneys, but any organ may be affected during the course of the illness. It may be difficult to diagnose, especially when c-ACNA antibodies (serologic symptom of Wegener's granulomatosis) are undetectable and chest X-ray is normal. Early diagnosis is crucial for treatment results. Untreated disease may lead to death. Cyclophosphamide used simultaneously with prednisone is the treatment of choice as the first line procedure. Resistance to standard systemic treatment may be a significant problem. New drugs (rituximab, infliximab) are still under clinical investigation, with promising results. Very limited data concerning effectiveness of radiation therapy exist. We present a report of a female patient with solitary form of Wegener's granulomatosis located in the facial region, who underwent successful radiation therapy with a complete response.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Low dose radiotherapy as an effective treatment in a patient with solitary Wegener’s granulomatosis resistant to systemic treatment –

Wygoda¹,

Rutkowski²,

Składowski³

et al. 2013

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“…Other novel therapies include etoposide, 35 15-deoxyspergualin, 36,37 intravenous azathioprine, 38 radiation therapy, 39,40 and high-dose myeloablative chemotherapy with stem cell transplantation. 41 Most of these methods, however, are associated with strong adverse effects.…”

Section: Othersmentioning

confidence: 99%

ANCA-associated Vasculitis: Diagnostic Therapeutic Strategy

Ozaki

2007

Allergology International

120

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Among small-vessel vasculitides, microscopic polyangiitis (MPA), Wegener's granulomatosis (WG), and allergic granulomatous angiitis (AGA) are known collectively as ANCA-associated vasculitis (AAV) because of the involvement of anti-neutrophil cytoplasmic antibodies (ANCA) as the common pathogenesis. Major target antigens of ANCA associated with vasculitis are myeloperoxidase (MPO) and proteinase 3 (PR3). MPO-ANCA is related to MPA and AGA, and PR3-ANCA is the marker antibody in WG. MPO-ANCA-associated vasculitis is more frequent in Japan, whereas PR3-ANCA-associated vasculitis is more common in Europe and USA. ANCA appears to induce vasculitis by directly activating neutrophils. Therefore, no immunoglobulins or complement components are detected in the vasculitis lesions; hence, AAV is called pauci-immune vasculitis (pauci = few/little). Untreated patients with severe AAV with multi-organ involvement have a poor prognosis, which is improved by combination therapy with cyclophosphamide and high-dose corticosteroid. Randomized controlled trials (RCT) regarding induction and maintenance of remission of AAV indicated that the rate of remission induction by the standard regimen is approximately 90% in 6 months, that maintenance of remission can be achieved with oral azathioprine as well as cyclophosphamide, and that methotrexate can be used only for non-renal mild AAV. As these data were obtained mostly in patients positive for PR3-ANCA, caution must be taken in applying these findings to Japanese patients, most of whom are positive for MPO-ANCA. A prospective study is now underway to clarify the effectiveness of the standard regimen in Japanese patients with MPO-ANCA-associated vasculitis. This article describes the diagnostic criteria and the recent evidence-based therapeutic strategy of AAV.

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“…Fortunately, the survival has been significantly improved with the instigation of combination therapies using cyclophosphamide and corticosteroids, with a mean five-year survival rate now of 90%-95% [63] and a mean survival rate of 21.7 years [64] . Airway involvement in WG is found in 15%-60% of patients but is rarely a presenting feature of the disease for most patients since laryngeal and trachea-bronchial involvements are usually late manifestations of the disease (Figure 18) [68][69][70][71][72][73][74][75] . Bronchoscopic findings include subglottic stenosis, ulcerating tracheobronchitis with or without inflammatory polyps or pseudotumors, and tracheal and bronchial stenosis [67,68] .…”

Section: Resultsmentioning

confidence: 99%

Modern imaging of the tracheo-bronchial tree

Laroia

Thompson²,

Laroia³

et al. 2010

WJR

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Recent state-of-the-art computed tomography and improved three-dimensional (3-D) postprocessing techniques have revolutionized the capability of visualizing airway pathology, offering physicians an advanced view of pathology and allowing for appropriate management planning. This article is a comprehensive review of trachea and main bronchi imaging, with emphasis on the dynamic airway anatomy, and a discussion of a wide variety of diseases including, but not limited to, congenital large airway abnormalities, tracheobronchial stenoses, benign and malignant neoplasms and tracheobronchomalacia. The importance of multiplanar reconstruction, 3-D reconstruction and incorporation of dynamic imaging for non-invasive evaluation of the large airways is stressed.

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Radiation Therapy and Mechanical Dilation of Endobronchial Obstruction Secondary to Wegener's Granulomatosis

Cited by 19 publications

References 4 publications

Low dose radiotherapy as an effective treatment in a patient with solitary Wegener’s granulomatosis resistant to systemic treatment –

Low dose radiotherapy as an effective treatment in a patient with solitary Wegener’s granulomatosis resistant to systemic treatment –

ANCA-associated Vasculitis: Diagnostic Therapeutic Strategy

Modern imaging of the tracheo-bronchial tree

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