Abstract
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.
Highlights
Granulomatosis with polyangiitis (GPA), previously referred to as Wegener’s granulomatosis, is a rare, idiopathic, systemic, auto-inflammatory disease
We have presented the case of a 69-year-old patient with acute-onset abdominal pain caused by GPAassociated peri-iliac retroperitoneal fibrosis resulting in a compression of local structures and hydronephrosis
Some case reports of GPA and Churg-Strauss syndrome causing retroperitoneal fibrosis have been published in recent years describing the association between the condition and the diseases [13]
Summary
Granulomatosis with polyangiitis (GPA), previously referred to as Wegener’s granulomatosis, is a rare, idiopathic, systemic, auto-inflammatory disease. Diagnosis remains a challenge because of the different nonspecific clinical manifestations patients present with during the early stages of the disease. These include, but are not limited to, rhinorrhea, oral ulcers, chronic sinusitis, and dyspnea [1]. Literature shows rare cases of GPA affecting large vessels and other structures such as the aorta and retroperitoneal tissue. These changes are often overlooked during clinical evaluation because of their rarity, but the associated risks of dissection or obstruction of local structures by the inflammation make an early diagnosis paramount. PET-CT: positron emission tomography-computed tomography; MIP: maximum intensity projection
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