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Case Reports
. 2016 Aug 26:19:121-4.
doi: 10.1016/j.rmcr.2016.08.009. eCollection 2016.

Retroperitoneal fibrosis with periaortitis: A case report of an unusual form of presentation of granulomatosis with polyangiitis

Elizabeth Manuely González Revilla  1 , Araceli Abad Fernandez  1 , María Teresa Río Ramirez  1 , Sara Calero Pardo  1 , María Antonia Juretschke Moragues  1
Affiliations
Case Reports

Retroperitoneal fibrosis with periaortitis: A case report of an unusual form of presentation of granulomatosis with polyangiitis

Elizabeth Manuely González Revilla et al. Respir Med Case Rep. .

Abstract

Granulomatosis with polyangiitis (GPA) is the name that has been used in recent years for Wegener's granulomatosis. This condition is a systemic inflammatory disease characterised by necrotizing vasculitis that affects small and medium-sized blood vessels (capillaries, arterioles, venules and arteries). The granulomatous inflammation affects the respiratory system; it also commonly affects the kidney and can very rarely affect large vessels such as the aorta and the surrounding retroperitoneal tissue. Early diagnosis and treatment is of vital importance because of the high risk of dissection and of obstruction of retroperitoneal structures. We present the case of a 74-year-old man with a past history of infrarenal abdominal aortic aneurysm. He consulted for abdominal pain. Cavitating pulmonary nodules and retroperitoneal fibrosis with periaortic alterations were detected on computed tomography. Laboratory investigations revealed that the patient was positive for cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) and necrotizing granulomas were observed on biopsies of the lung lesions and retroperitoneal tissue. The patient was diagnosed with GPA and treatment was started with glucocorticoids and immunosuppressive agents, which led to a significant clinical and radiological improvement over the following months.

Keywords: C. ANCA-Positive periaortic vasculitis; Retroperitoneal fibrosis.

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Figures

Fig. 1
Fig. 1
A, Multiple bilateral cavitating pulmonary lesions. B, Image 3 months after treatment: there is a reduction in the overall size of the nodular pulmonary lesions, with residual millimetric changes.
Fig. 2
Fig. 2
Positron emission tomography–computed tomography. A, Pathological increase in glucose metabolism in the soft tissue mass surrounding the infrarenal abdominal aorta. B, Bilateral hypermeabolic pulmonary modules.
Fig. 3
Fig. 3
Biopsy of abdominal para-aortic mass. Acute inflammation and chronic granulomatous suppurative necrotizing.
See this image and copyright information in PMC

References

    1. Langford C.A., Talar-Williams C., Sneller M.C. Use of methotrexate and glucocorticoids in the treatment of Wegener's granulomatosis. Long-term renal outcome in patients with glomerulonephritis. Arthritis Rheum. 2000;43:1836. - PubMed
    1. Mavragani Clio P., Voulgarelis Michalis. Retroperitoneal fibrosis and CANCA positivity received. Clin. Rheumatol. 2007;26:115–116. - PubMed
    1. Van Bommel E., Brouwers A., Makkus A., Van Vliet A. Retroperitoneal fibrosis and p-ANCA-associated polyarteritis nodosa: coincidental or common etiology? Eur. J. Intern Med. 2002;13:392. - PubMed
    1. Vaglio A., Manenti L., Allegri L., Ferrozzi F., Corradi D., Buzio C. ANCApositive periaortic vasculitis: does it fall within the spectrum of vasculitis? J. Intern Med. 2002;251:268–271. - PubMed
    1. Kaipiainen-Seppanen O., Jantunen E., Kuusisto J., Marin S. Retroperitoneal fibrosis with antineutrophil cytoplasmic antibodies. J. Rheumatol. 1996;23:779–781. - PubMed

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