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A Rare Case of Granulomatosis With Polyangiitis Presenting as Retroperitoneal Fibrosis in the Peri-Iliac Region Causing Hydronephrosis

Wegener’s granulomatosis, now more commonly referred to as granulomatosis with polyangiitis (GPA), is a rare, idiopathic, systemic inflammatory disease, most commonly involving the respiratory tract, kidneys, and sinonasal region. The condition affects small and medium-sized blood vessels, such as a...

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Autores principales: Šoštarič, Kristijan, Lovrec Krstić, Tina, Slanič, Aleš, Caf, Primož
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8451527/
https://www.ncbi.nlm.nih.gov/pubmed/34567857
http://dx.doi.org/10.7759/cureus.17295
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author Šoštarič, Kristijan
Lovrec Krstić, Tina
Slanič, Aleš
Caf, Primož
author_facet Šoštarič, Kristijan
Lovrec Krstić, Tina
Slanič, Aleš
Caf, Primož
author_sort Šoštarič, Kristijan
collection PubMed
description Wegener’s granulomatosis, now more commonly referred to as granulomatosis with polyangiitis (GPA), is a rare, idiopathic, systemic inflammatory disease, most commonly involving the respiratory tract, kidneys, and sinonasal region. The condition affects small and medium-sized blood vessels, such as arteries, arterioles, venules, and capillaries. Some cases of the disease presenting as retroperitoneal fibrosis and/or affecting the aorta have been reported. Although advances in the treatment of GPA have contributed to a decline in mortality, early diagnosis is still of vital importance due to the possible complications of the disease. Here, we present the case of a 69-year-old man with acute-onset abdominal pain. Ultrasound of the abdomen showed left-sided hydronephrosis. Computed tomography detected cavitating pulmonary lesions and peri-iliac alterations caused by retroperitoneal fibrosis with involvement of the crossing ureter. Laboratory results revealed high antineutrophil cytoplasmic antibody levels and high inflammatory markers. A lung biopsy performed via bronchoscopy revealed necrotizing granulomas and solidified the diagnosis of GPA both in the lung and the peri-iliac region. Treatment with immunosuppressive agents and glucocorticoids was initiated. A follow-up after two months showed regression of the pulmonary lesions and partial resolution of the hydronephrosis as well as reduced inflammatory markers in the blood tests.
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spelling pubmed-84515272021-09-23 A Rare Case of Granulomatosis With Polyangiitis Presenting as Retroperitoneal Fibrosis in the Peri-Iliac Region Causing Hydronephrosis Šoštarič, Kristijan Lovrec Krstić, Tina Slanič, Aleš Caf, Primož Cureus Internal Medicine Wegener’s granulomatosis, now more commonly referred to as granulomatosis with polyangiitis (GPA), is a rare, idiopathic, systemic inflammatory disease, most commonly involving the respiratory tract, kidneys, and sinonasal region. The condition affects small and medium-sized blood vessels, such as arteries, arterioles, venules, and capillaries. Some cases of the disease presenting as retroperitoneal fibrosis and/or affecting the aorta have been reported. Although advances in the treatment of GPA have contributed to a decline in mortality, early diagnosis is still of vital importance due to the possible complications of the disease. Here, we present the case of a 69-year-old man with acute-onset abdominal pain. Ultrasound of the abdomen showed left-sided hydronephrosis. Computed tomography detected cavitating pulmonary lesions and peri-iliac alterations caused by retroperitoneal fibrosis with involvement of the crossing ureter. Laboratory results revealed high antineutrophil cytoplasmic antibody levels and high inflammatory markers. A lung biopsy performed via bronchoscopy revealed necrotizing granulomas and solidified the diagnosis of GPA both in the lung and the peri-iliac region. Treatment with immunosuppressive agents and glucocorticoids was initiated. A follow-up after two months showed regression of the pulmonary lesions and partial resolution of the hydronephrosis as well as reduced inflammatory markers in the blood tests. Cureus 2021-08-18 /pmc/articles/PMC8451527/ /pubmed/34567857 http://dx.doi.org/10.7759/cureus.17295 Text en Copyright © 2021, Šoštarič et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Internal Medicine
Šoštarič, Kristijan
Lovrec Krstić, Tina
Slanič, Aleš
Caf, Primož
A Rare Case of Granulomatosis With Polyangiitis Presenting as Retroperitoneal Fibrosis in the Peri-Iliac Region Causing Hydronephrosis
title A Rare Case of Granulomatosis With Polyangiitis Presenting as Retroperitoneal Fibrosis in the Peri-Iliac Region Causing Hydronephrosis
title_full A Rare Case of Granulomatosis With Polyangiitis Presenting as Retroperitoneal Fibrosis in the Peri-Iliac Region Causing Hydronephrosis
title_fullStr A Rare Case of Granulomatosis With Polyangiitis Presenting as Retroperitoneal Fibrosis in the Peri-Iliac Region Causing Hydronephrosis
title_full_unstemmed A Rare Case of Granulomatosis With Polyangiitis Presenting as Retroperitoneal Fibrosis in the Peri-Iliac Region Causing Hydronephrosis
title_short A Rare Case of Granulomatosis With Polyangiitis Presenting as Retroperitoneal Fibrosis in the Peri-Iliac Region Causing Hydronephrosis
title_sort rare case of granulomatosis with polyangiitis presenting as retroperitoneal fibrosis in the peri-iliac region causing hydronephrosis
topic Internal Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8451527/
https://www.ncbi.nlm.nih.gov/pubmed/34567857
http://dx.doi.org/10.7759/cureus.17295
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