An unusual presentation of limited granulomatosis with polyangiitis as a periurethral tumor

Amit Pande,Jyotsna Oak,Sameer Rajadhyaksha,Trideep Das,Vinita Salvi

doi:10.15305/ijrci/v9i1/344

Abstract

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.

Highlights

Granulomatosis with polyangiitis is an anti-neutrophil cytoplasmic antibody (ANCA)-associated multisystemic small vessel vasculitis
Even though Granulomatosis with polyangiitis (GPA) is a systemic vasculitis, some patients can present with limited form mainly involving respiratory tract or periorbital area.[2]
We report a case of GPA affecting urethra and vagina in the form of a pseudotumor in a young female

Summary

Introduction

Granulomatosis with polyangiitis is an anti-neutrophil cytoplasmic antibody (ANCA)-associated multisystemic small vessel vasculitis. Even though GPA is a systemic vasculitis, some patients can present with limited form mainly involving respiratory tract or periorbital area.[2] Limited urogenital tract form is observed in

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Discussion

Conclusion