CC BY-NC-ND 4.0 · Ann Natl Acad Med Sci 2023; 59(01): 055-059
DOI: 10.1055/s-0042-1760352
Case Report

Granulomatosis with Polyangiitis Presenting as a Renal Mass: A Scarce Case Report with a Review of the Literature

1   Department of Urology, IGMC, Shimla, Himachal Pradesh, India
,
Kailash Chander Barwal
1   Department of Urology, IGMC, Shimla, Himachal Pradesh, India
,
Sunish Sharma
2   Department of Pathology, IGMC, Shimla, Himachal Pradesh, India
,
Sanjeev Chauhan
1   Department of Urology, IGMC, Shimla, Himachal Pradesh, India
,
Pamposh Raina
1   Department of Urology, IGMC, Shimla, Himachal Pradesh, India
› Author Affiliations

Abstract

Wegener granulomatosis (WG) now known as granulomatosis with polyangiitis (GPA) is an uncommon autoimmune disorder of undivulged etiology affecting the respiratory tract including paranasal sinuses, nasal cavity, lungs, and kidneys predominantly. GPA presenting as a solitary renal mass is rarely seen. We present a case report of a 27-year-old female presenting with a right renal mass along with pain, low-grade fever, and arthralgia. Computed tomography scan of the abdomen revealed a hypodense low attenuated renal mass with indistinct margins. Ultrasound-guided biopsy revealed features typical of GPA. She was started on oral steroids (prednisolone 40 mg) and azathioprine. She developed pain, vomiting, and diarrhea after starting treatment with azathioprine. Azathioprine was stopped and rituximab 1 g weekly was started for 4 weeks followed by 500 mg 6 monthly injections. She got symptomatic relief at 4 weeks with a diminution of renal mass at 6 months follow-up. We report this rare entity of WG presenting as renal mass. Suspecting and diagnosing renal mass as a part of GPA prevented us from undertaking unnecessary surgical treatment in this patient. Medical treatment with steroids and rituximab is effective in inducing remission and maintenance.

Granulomatosis with polyangitis is uncommon autoimmune small to medium vessel disease affecting kidney frequently. GPA presenting as solitary renal mass is very rarely seen. Early diagnosis of GPA on basis of clinical features and radiological study like CT scan is crucial. Imaging guided biopsy is paramount for making a diagnosis of GPA. Diagnosing GPA could prevent surgery as medical treatment is effective. We highlight our rare presentation of GPA with renal mass and its medical management.




Publication History

Article published online:
16 February 2023

© 2023. National Academy of Medical Sciences (India). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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