The patient with Wegener's granulomatosis and an intrasplenic mass of unknown origin

Hafezi‐Rachti, Syrus; Riess, R; Weidner, Sven; Wonka, Andrea; Rupprecht, Harald

doi:10.1093/ndt/15.6.906

Cited by 5 publications

(2 citation statements)

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“…Hafezi-Rachti et al 3 illustrated a case of near complete splenic infarct from WG managed conservatively, with scarring of the infarcted spleen demonstrated on a repeat CT scan 2.5 years later. In our patient, splenectomy was performed because the concurrent sepsis posed increased risk of splenic abscess.…”

Section: Discussionmentioning

confidence: 99%

Asymptomatic splenic infarct and retroperitoneal sepsis in patient with Wegener's granulomatosis

Lee

Bringmann

Aly

2012

International Journal of Surgery Case Reports

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Section: Discussionmentioning

confidence: 99%

Asymptomatic splenic infarct and retroperitoneal sepsis in patient with Wegener's granulomatosis

Lee

Bringmann

Aly

2012

International Journal of Surgery Case Reports

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“…However, isolated case reports of patients with WG and incomplete septal cirrhosis, 184 primary biliary cirrhosis, 185 and portal inflammatory granuloma, vasculitis, and fibrosis 186 have been published. Splenic involvement is rarely diagnosed antemortem, [187][188][189][190] but splenic infarcts have been documented at necropsies 191,192 or on abdominal CT (low attenuation lesions with peripheral enhancement). 187,193 OTHER ORGAN INVOLVEMENT Constitutional symptoms (e.g., malaise, fatigue, fever, weight loss) occur in 30 to 80% of patients with WG and may be the presenting features.…”

Section: Gastrointestinal Involvementmentioning

confidence: 99%

Wegener's Granulomatosis: Evolving Concepts in Treatment

Lynch

White

Tazelaar

et al. 2004

Seminars in Respiratory and Critical Care Medicine

171

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Wegener's granulomatosis (WG), the most common of the pulmonary granulomatous vasculitides, typically involves the upper respiratory tract, lower respiratory tract (bronchi and lung), and kidney, with varying degrees of disseminated vasculitis. Major histological features include a necrotizing vasculitis involving small vessels, extensive "geographic" necrosis, and granulomatous inflammation. Clinical manifestations of WG are protean; virtually any organ can be involved. Further, the spectrum and severity of the disease is heterogeneous, ranging from indolent disease involving only one site to fulminant, multiorgan vasculitis leading to death. The pathogenesis of WG has not been elucidated, but both cellular and humoral components are involved. Circulating antineutrophil cytoplasmic antibodies (cANCA) likely play a role in the pathogenesis and often correlate with activity of the disease. Treatment strategies are evolving. Cyclophosphamide (CYC) plus corticosteroids (CS) is the mainstay of therapy for generalized, multisystemic WG. Historically, the combination of CYC plus CS was used for a minimum of 12 months, but concern about late toxicities associated with CYC has led to novel treatment approaches. Currently, short-course (3-6 months) induction treatment with CYC plus CS, followed by maintenance therapy with less toxic agents (e.g., methotrexate, azathioprine) is recommended. Further, recent studies suggest that methotrexate combined with CS may be adequate for limited, non-life threatening WG. The role of other immunomodulatory agents (including trimethoprim-sulfamethoxazole) is also explored.

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Endocarditis associated with antineutrophil cytoplasmic antibodies: a case report and review of the literature

et al. 2006

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We report a case of subacute bacterial endocarditis associated with small vessel vasculitis and a strongly positive cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) test. It is important to recognize this cause of positive c-ANCA because infectious endocarditis may closely mimic the clinical manifestations of ANCA-associated vasculitides such as Wegener granulomatosis or microscopic polyangiitis. Furthermore, ANCA-associated vasculitis may result in noninfectious endocarditis, which may be confused with bacterial endocarditis. In this paper, we review reported cases of ANCA-positive bacterial endocarditis and compare them to the reported cases of ANCA-associated idiopathic vasculitis with endocardial compromise.

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The patient with Wegener's granulomatosis and an intrasplenic mass of unknown origin

Cited by 5 publications

References 1 publication

Asymptomatic splenic infarct and retroperitoneal sepsis in patient with Wegener's granulomatosis

Asymptomatic splenic infarct and retroperitoneal sepsis in patient with Wegener's granulomatosis

Wegener's Granulomatosis: Evolving Concepts in Treatment

Endocarditis associated with antineutrophil cytoplasmic antibodies: a case report and review of the literature

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