Retroperitoneal fibrosis and p-ANCA-associated polyarteritis nodosa: coincidental or common etiology?

Bommel, E. F. H. van; Brouwers, A.J.W.B; Makkus, L.; Vliet, A. C. M. Van

doi:10.1016/s0953-6205(02)00096-1

Cited by 15 publications

(7 citation statements)

References 10 publications

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“…Although no possible causes of MF were identified in the present case, quite interestingly, the patient was diagnosed as having ANCA-associated nephritis coinciding with mediastinal disease. Given that ANCA have been reported to be a possible etiology for RPF (3)(4)(5), the patient's MF may have been associated with ANCA positivity. An association between MF and ANCA has been speculated because the occurrence of vasculitis associated with fibrosis has been reported in some patients with RPF; such cases may well have begun as vasculitis, inducing a chronic inflammatory reaction and subsequent fibroblast proliferation (2,3,8).…”

Section: Discussionmentioning

confidence: 95%

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Mediastinal Fibrosis and Positive Antineutrophil Cytoplasmic Antibodies: Coincidence or Common Etiology?

et al. 2014

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Retroperitoneal fibrosis (RPF) with positive antineutrophil cytoplasmic antibodies (ANCA) has been reported in several cases. We herein present the case of a 52-year-old woman who was diagnosed with mediastinal fibrosis (MF) on a thoracoscopic surgical biopsy. The patient had positive myeloperoxidase ANCA and thereafter developed crescentic glomerulonephritis, which was considered to be a form of ANCA-related nephritis. Both the MF and crescentic glomerulonephritis favorably responded to immunosuppressive therapy. These findings suggest a common pathogenesis of these disorders involving ANCA positivity, as reported in patients with RPF.

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Section: Discussionmentioning

confidence: 95%

“…A wide variety of factors are possible causes of this disease, as in MF. In addition, antineutrophil cytoplasmic antibodies (ANCA) have been reported to be a possible etiology for RPF, although the precise pathomechanism has not been proven (3)(4)(5).…”

Section: Introductionmentioning

confidence: 99%

Mediastinal Fibrosis and Positive Antineutrophil Cytoplasmic Antibodies: Coincidence or Common Etiology?

et al. 2014

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“…8 A possible link between RPF, ANCA and small-vessel vasculitis is not surprising when we consider that about 10% of RPF masses contain overt vasculitic lesions with fibrinoid necrosis in the small vessels. 11 Furthermore, recent case reports have described isolated RPF associated with positive C-or P-ANCA 12,13 and RPF associated with defined systemic vasculitis, including Takayasu arteritis 14 and c-ANCA or p-ANCA-positive vasculitis, such as periarteritis nodosa, 15 Churg-Strauss syndrome 16 and Wegener's granulomatosis. 17 Before the availability of ANCAtesting, other cases of RPF associated with vasculitis were reported in which vasculitis involved the small vessels.…”

Section: Discussionmentioning

confidence: 99%

Wegener’s granulomatosis and retroperitoneal fibrosis: a case report and review of the literature

et al. 2019

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Granulomatosis with polyangiitis (GPA), also known as Wegener’s granulomatosis (WG), is a rare systemic auto-inflammatory disease characterized by necrotizing granulomatous inflammation and antineutrophil cytoplasmic antibodies-associated small vessel vasculitis. Retroperitoneal fibrosis (RPF) is an uncommon collagen vascular disease of unclear etiology, characterized by a chronic non-specific inflammation of the retroperitoneum, which can entrap and obstruct retroperitoneal structures, notably abdominal aorta, iliac arteries and ureters. RPF is mostly idiopathic, but an association with vasculitis has been shown. However, only few cases of retroperitoneal fibrosis with ureterohydronephrosis secondary to GPA have been described in literature. The outcome seems to be better when compared to the idiopathic form, and immunosuppressive treatment demonstrated to be effective, with low need in surgery. We provide the case report of a 57-year-old patient, affected by GPA, referred to our Division for massive vein thrombosis and ureteral stenosis secondary to retroperitoneal fibrosis. We also provide a review of the literature currently available on this topic.

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“…The association of retroperitoneal fibrosis with c-ANCA or p-ANCA–positive systemic vasculitis is described in the literature, not only in granulomatosis with polyangiitis but also in other vasculitides such as Churg-Strauss syndrome [17] . A good response to immunosuppressive treatment with cyclophosphamide and corticosteroids has been reported [3] , [4] , [5] , [6] . The autoimmune nature of the disease added to inflammatory changes affecting small and medium-sized vessels would explain the clinical manifestations of the retroperitoneal fibrosis.…”

Section: Discussionmentioning

confidence: 99%

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

Revilla

Fernández

Ramírez

et al. 2016

Respiratory Medicine Case Reports

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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.

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Retroperitoneal fibrosis and p-ANCA-associated polyarteritis nodosa: coincidental or common etiology?

Cited by 15 publications

References 10 publications

Mediastinal Fibrosis and Positive Antineutrophil Cytoplasmic Antibodies: Coincidence or Common Etiology?

Mediastinal Fibrosis and Positive Antineutrophil Cytoplasmic Antibodies: Coincidence or Common Etiology?

Wegener’s granulomatosis and retroperitoneal fibrosis: a case report and review of the literature

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

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