An unusual presentation of limited granulomatosis with polyangiitis as a periurethral tumor
DOI:
https://doi.org/10.15305/ijrci/v9i1/344Keywords:
Granulomatosis with polyangitis, GPA, ANCAAbstract
Granulomatosis with polyangitis (GPA) is a systemic necrotizing granulomatous vasculitis characterized by affection of small and medium sized blood vessels .It normally affects upper and lower respiratory tract and Kidneys in most of the cases . Genital involvement in GPA is rare and is reported in less than 1% of cases. It is seen along with other systemic involvement and in patients who are already diagnosed with GPA. We present a case of a young girl with limited GPA ,who presented initially as an irregular mass occupying vagina and periurethral area .The diagnosis was reached on histopathology and patient showed good response only after corticosteroids and cyclophosphamide.References
Jennette JC,Falk RJ, Bacon PA, et al, 2012 Revised International Chapel Hill Consensus Conference Nomencleture of Vasculitidis.Arthritis Rheum 2013:65:1-11
Bastone P, Squifflet JL, Marbaix E and Houssiau F Successful treatment of gynaecological involvement of granulomatosis with polyangitis (Wegners granulomatosis) by rituximab, Clinical and Experimental Rheumatoloy, vol.33, no. 2. Supplement 89, pp.S-142-S-144,2015.
Alba MA, Morenco-Palacios J, Beca S and Cid MC, Urologic and male genital manifestations of granulomatosis with polyangitis Autoimmunity Reviews, vol 14,no. 10 pp897-902,2015
Dufour JF, Le Gallou T, Cordier JF et al, Urogenital manifestations in Wegners granulomatosis: a study of 11 cases and review of the literature, Medicine, vol 91, no.2 pp. 67-74,2012
Huong DLT,PapoT, Piette JC et al Urogenital manifestations of Wegners granulomatosis, Medicine (united States), vol 74, no.3.pp.152-161,1995.
Davenport A, Dawney SE,Goel S and Maclvert AG,Wegners granulomatosis involving the urogenital tract British Journal of Urology , vol 78, no 3 pp 354-357,1996.
Ward A,Konya C Mark EJ and Rosen S, Granulomatosis with polyangitis presenting as a renal tumor American Journal of Surgical Pathology, vol 38, no. 10 pp.1444-1448,2014.
Roussou M, Dimopoulos SK, Dimopoulos MA, Anastasiou-Nana MI, Wegners granulomatosis presenting as a renal mass, Urology vol 1, no. 3. Pp 547.el -547 e2,2008.
Ruiz Carazo E, Medina Benitez A, Lopez Milena G, Rabaza Espigares J, Leon L and Marquez B Multiple renal masses as initial manifestation of Wegners Granulomatosis , American Journal of Roentgenology, vol 176, no.1.pp.116-118, 2001.
Villa Forte A and Hoffman GS,Wegners Granulomatosis presenting with a renal mass , Journal of Rheumatology, vol 26,no,2, pp. 457-458,1999.
Smith DJ, Milroy M, and Chapple CR, , An unusual renal mass? Wegners Granulomatosis, British Journal of Urology, vol 72, pp.980-981,1993.
Published
Issue
Section
License
Copyright for articles published in this journal is retained by the authors, with first publication rights granted to the ‘Internet Journal of Rheumatology and Clinical Immunology’. All the published content, except where otherwise noted, is licensed under Creative Commons license (CC-BY).
Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) after the publication of the manuscript in IJRCI, as it can lead to productive exchanges, as well as earlier and greater citation of published work.
