Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2017:2017:9407675.
doi: 10.1155/2017/9407675. Epub 2017 Mar 13.

An Unusual Presentation of Limited Granulomatosis with Polyangiitis Involving Vagina and Urethra

Sandra Soro Marín  1 , Enrique Júdez Navarro  2 , Manuela Sianes Fernández  2 , Ginés Sánchez Nievas  2 , Juan Gabriel Lorenzo Romero  3
Affiliations
Case Reports

An Unusual Presentation of Limited Granulomatosis with Polyangiitis Involving Vagina and Urethra

Sandra Soro Marín et al. Case Rep Rheumatol. 2017.

Abstract

Granulomatosis with polyangiitis (GPA) is a systemic necrotizing granulomatosis vasculitis characterized by predilection to affect small- and medium-sized blood vessels and commonly affects the upper and lower respiratory tract and kidneys in most cases. Genital involvement is reported in <1% of cases in large cohorts and nearly all cases have been in the setting of multisystemic disease or during the course of the disease in patients already diagnosed as GPA. A case is presented of uncommon limited urogenital GPA in a 66-year-old woman with an irregular mass occupying urethra and vagina. The patient showed a good response after Corticoids and Methotrexate.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Coronal plane pelvic MRI showed an irregular mass with crenulated margins occupying the urethra and vagina.
Figure 2
Figure 2
Axial plane pelvic MRI showed an irregular mass with crenulated margins occupying the urethra and vagina.
See this image and copyright information in PMC

References

    1. Jennette J. C., Falk R. J., Bacon P. A., et al. 2012 Revised International Chapel Hill consensus conference nomenclature of vasculitides. Arthritis and Rheumatism. 2013;65(1):1–11. doi: 10.1002/art.37715. - DOI - PubMed
    1. Bastone P., Squifflet J. L., Marbaix E., Houssiau F. Successful treatment of gynaecological involvement of granulomatosis with polyangiitis (Wegener's granulomatosis) by rituximab. Clinical and Experimental Rheumatology. 2015;33(2, supplement 89):S-142–S-144. - PubMed
    1. Alba M. A., Moreno-Palacios J., Beça S., Cid M. C. Urologic and male genital manifestations of granulomatosis with polyangiitis. Autoimmunity Reviews. 2015;14(10):897–902. doi: 10.1016/j.autrev.2015.05.012. - DOI - PubMed
    1. Comarmond C., Cacoub P. Granulomatosis with polyangiitis (Wegener): clinical aspects and treatment. Autoimmunity Reviews. 2014;13(11):1121–1125. doi: 10.1016/j.autrev.2014.08.017. - DOI - PubMed
    1. Dufour J.-F., Le Gallou T., Cordier J.-F., et al. Urogenital manifestations in Wegener granulomatosis: a study of 11 cases and review of the literature. Medicine. 2012;91(2):67–74. doi: 10.1097/md.0b013e318239add6. - DOI - PubMed

Publication types

LinkOut - more resources