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

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.

Granulomatosis with Polyangiitis-Mimicking Advanced Gynecological Cancer: A Case Report and Systematic Review of the Literature

“…A higher recurrence rate is observed with a diagnosis delay of 12 months or longer. However, there is not a single patient with genital involvement and without a uterus (previous hysterectomy or performed at diagnosis) who has experienced a recurrence, including our patient [14,15,18,19].…”

“…Our systematic review identified 19 patients with GPA and genital tract involvement [12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28]. Among them 11/19 (58%) had primary disease, and 8/19 (42%) had recurrence.…”

“…The most common clinical presentation of primary GPA is that of a gynecologic malignancy, as noted in 68% of the studies reviewed [2,[16][17][18][19][22][23][24][26][27][28][29][30]. Some of these patients received incorrect treatments such as surgical excision, anti-tuberculosis treatment, and/or radiotherapy [13][14][15]18,20,21,24,28].…”

“…Significant morbidity results from the disease and its treatment, such end-stage renal disease and adrenal or multiorgan failure [12,[19][20][21]24,26,29]. As indicate above the mortality rate of gynecological disease is 16% [15,21,24], as compared to slightly higher than 25% [2].…”

“…Once studies with patients with MPA or EGPA (5) were removed, a total of 17 studies fulfilled all inclusion criteria and were selected for full-text evaluation and systematic review [12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28]. After reviewing the 17 articles, 18 patients were identified as GPA with gynecological involvement (two patients reported by Stone et al) [12].…”