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

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 cou...

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Autores principales: Wygoda, Andrzej, Rutkowski, Tomasz, Składowski, Krzysztof, Hejduk, Beata
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Termedia Publishing House 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3685357/
https://www.ncbi.nlm.nih.gov/pubmed/23788974
http://dx.doi.org/10.5114/wo.2013.33785
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author Wygoda, Andrzej
Rutkowski, Tomasz
Składowski, Krzysztof
Hejduk, Beata
author_facet Wygoda, Andrzej
Rutkowski, Tomasz
Składowski, Krzysztof
Hejduk, Beata
author_sort Wygoda, Andrzej
collection PubMed
description 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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spelling pubmed-36853572013-06-20 Low dose radiotherapy as an effective treatment in a patient with solitary Wegener's granulomatosis resistant to systemic treatment – case report Wygoda, Andrzej Rutkowski, Tomasz Składowski, Krzysztof Hejduk, Beata Contemp Oncol (Pozn) Case Report 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. Termedia Publishing House 2013-03-15 2013 /pmc/articles/PMC3685357/ /pubmed/23788974 http://dx.doi.org/10.5114/wo.2013.33785 Text en Copyright © 2013 Termedia http://creativecommons.org/licenses/by-nc-nd/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution-Noncommercial 3.0 Unported License, permitting all non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Wygoda, Andrzej
Rutkowski, Tomasz
Składowski, Krzysztof
Hejduk, Beata
Low dose radiotherapy as an effective treatment in a patient with solitary Wegener's granulomatosis resistant to systemic treatment – case report
title Low dose radiotherapy as an effective treatment in a patient with solitary Wegener's granulomatosis resistant to systemic treatment – case report
title_full Low dose radiotherapy as an effective treatment in a patient with solitary Wegener's granulomatosis resistant to systemic treatment – case report
title_fullStr Low dose radiotherapy as an effective treatment in a patient with solitary Wegener's granulomatosis resistant to systemic treatment – case report
title_full_unstemmed Low dose radiotherapy as an effective treatment in a patient with solitary Wegener's granulomatosis resistant to systemic treatment – case report
title_short Low dose radiotherapy as an effective treatment in a patient with solitary Wegener's granulomatosis resistant to systemic treatment – case report
title_sort low dose radiotherapy as an effective treatment in a patient with solitary wegener's granulomatosis resistant to systemic treatment – case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3685357/
https://www.ncbi.nlm.nih.gov/pubmed/23788974
http://dx.doi.org/10.5114/wo.2013.33785
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