The association between autoimmune disease and risk of monoclonal malignancy is well studied. Howeven monoclonal B-cell lymphocytosis （MBL） in patients with autoimmune diseases has rarely been reported. The newly pu...The association between autoimmune disease and risk of monoclonal malignancy is well studied. Howeven monoclonal B-cell lymphocytosis （MBL） in patients with autoimmune diseases has rarely been reported. The newly published 2016 revision of the World Health Organization （WHO） classification of lymphoid neoplasms has officially accepted MBL as an independent disease entity Herein, we present a case of Wegener granulomatosis （WG） with MBL.展开更多
A case is presented of Wegener＇s granulomatosis limited to the testis and epididymis, simultaneously, in a 69-year-old man. Orchiectomy was carried out through an inguinal incision under the presumptive diagnosis of ...A case is presented of Wegener＇s granulomatosis limited to the testis and epididymis, simultaneously, in a 69-year-old man. Orchiectomy was carried out through an inguinal incision under the presumptive diagnosis of a right testicular tumor. A hard, irregular mass occupied the upper testicle and a portion of the epididymal head was visualized. Histopathologic examination of the specimen showed granulomatous inflammation of the testis and epididymis with prominent angiocentric granulomata in the walls of arteries, veins and foci of fibrinoid necrosis, surrounded by palisading inflammatory cells with a few giant cells. The diagnosis of limited Wegener＇s granulomatosis was considered, although antineutrophil cytoplasmic antibody （c-ANCA） test was negative 2 weeks after orchiectomy. The patient showed an excellent response after local complete excision. He remains free of disease 18 months after orchiectomy.展开更多
BACKGROUND Granulomatosis with polyangiitis(GPA)is a rare autoimmune disease that involves small-to-medium-sized vessels and forms necrotizing vasculitis with granulomatous inflammation.The formation of a large vessel...BACKGROUND Granulomatosis with polyangiitis(GPA)is a rare autoimmune disease that involves small-to-medium-sized vessels and forms necrotizing vasculitis with granulomatous inflammation.The formation of a large vessel lesion in GPA patients has been scarcely reported,and it can cause confusion in the diagnosis.CASE SUMMARY A 27-year-old man presented with mild left-sided pleuritic chest pain that started one year prior.An imaging study revealed up to 2.5 cm-sized two irregular nodular consolidation nodule in the left lower lobe.Both nodules showed central necrosis.Also,there was a periaortic mass occluding the branching porting of the subclavian artery.He had positive anti-neutrophil cytoplasmic antibodies(ANCAs),but myeloperoxidase-ANCAs and proteinase 3-ANCAs were negative.The patient also developed symptoms of subclavian vein syndrome during the follow-up.Wedge resection of the lung revealed necrotizing vasculitis,destructive parenchymal abscess and surrounding granuloma,and therefore diagnosed of GPA.The patient started on methotrexate and steroid therapy with a relief of symptomatic.CONCLUSION Here,we present an unusual manifestation of GPA with periaortitis and consequent subclavian steal syndrome,which has never been previously described.This case alerts us that we should include GPA in the differential diagnosis of large vessel vasculitis as well as subclavian steal syndrome.展开更多
In part 1 of this article,clinical features and imaging findings of Takayasu arteritis(also known as aortitis syndrome)were described in detail.In part 2,treatment of Takayasu arteritis will be first described and dis...In part 1 of this article,clinical features and imaging findings of Takayasu arteritis(also known as aortitis syndrome)were described in detail.In part 2,treatment of Takayasu arteritis will be first described and discussed.This will be followed by description of clinical features and imaging findings of other systemic vasculitis.Comments on interventional radiology for systemic vasculitis will also be made.展开更多
文摘The association between autoimmune disease and risk of monoclonal malignancy is well studied. Howeven monoclonal B-cell lymphocytosis （MBL） in patients with autoimmune diseases has rarely been reported. The newly published 2016 revision of the World Health Organization （WHO） classification of lymphoid neoplasms has officially accepted MBL as an independent disease entity Herein, we present a case of Wegener granulomatosis （WG） with MBL.
文摘A case is presented of Wegener＇s granulomatosis limited to the testis and epididymis, simultaneously, in a 69-year-old man. Orchiectomy was carried out through an inguinal incision under the presumptive diagnosis of a right testicular tumor. A hard, irregular mass occupied the upper testicle and a portion of the epididymal head was visualized. Histopathologic examination of the specimen showed granulomatous inflammation of the testis and epididymis with prominent angiocentric granulomata in the walls of arteries, veins and foci of fibrinoid necrosis, surrounded by palisading inflammatory cells with a few giant cells. The diagnosis of limited Wegener＇s granulomatosis was considered, although antineutrophil cytoplasmic antibody （c-ANCA） test was negative 2 weeks after orchiectomy. The patient showed an excellent response after local complete excision. He remains free of disease 18 months after orchiectomy.
文摘BACKGROUND Granulomatosis with polyangiitis(GPA)is a rare autoimmune disease that involves small-to-medium-sized vessels and forms necrotizing vasculitis with granulomatous inflammation.The formation of a large vessel lesion in GPA patients has been scarcely reported,and it can cause confusion in the diagnosis.CASE SUMMARY A 27-year-old man presented with mild left-sided pleuritic chest pain that started one year prior.An imaging study revealed up to 2.5 cm-sized two irregular nodular consolidation nodule in the left lower lobe.Both nodules showed central necrosis.Also,there was a periaortic mass occluding the branching porting of the subclavian artery.He had positive anti-neutrophil cytoplasmic antibodies(ANCAs),but myeloperoxidase-ANCAs and proteinase 3-ANCAs were negative.The patient also developed symptoms of subclavian vein syndrome during the follow-up.Wedge resection of the lung revealed necrotizing vasculitis,destructive parenchymal abscess and surrounding granuloma,and therefore diagnosed of GPA.The patient started on methotrexate and steroid therapy with a relief of symptomatic.CONCLUSION Here,we present an unusual manifestation of GPA with periaortitis and consequent subclavian steal syndrome,which has never been previously described.This case alerts us that we should include GPA in the differential diagnosis of large vessel vasculitis as well as subclavian steal syndrome.
文摘In part 1 of this article,clinical features and imaging findings of Takayasu arteritis(also known as aortitis syndrome)were described in detail.In part 2,treatment of Takayasu arteritis will be first described and discussed.This will be followed by description of clinical features and imaging findings of other systemic vasculitis.Comments on interventional radiology for systemic vasculitis will also be made.
