BACKGROUND Vasculitis,a systemic disorder with inflammation of blood vessel walls,can develop broad spectrum of signs and symptoms according to involvement of various organs,and therefore,early diagnosis of vasculitis...BACKGROUND Vasculitis,a systemic disorder with inflammation of blood vessel walls,can develop broad spectrum of signs and symptoms according to involvement of various organs,and therefore,early diagnosis of vasculitis is challenging.We herein describe a patient who developed a special case of systemic vasculitis with mononeuropathy multiplex,rectal perforation and antiphospholipid syndrome(APS)presented with pulmonary embolism.CASE SUMMARY A 61-year-old woman visited hospital with complaints of myalgia and occasional fever.She was initially diagnosed as proctitis and treated with antibiotics,however,there was no improvement.In addition,she also complained right foot drop with hypesthesia,and left 2^(nd) and 3^(rd) finger tingling sensation.She underwent nerve conduction study for evaluation,and it revealed sensorimotor polyneuropathy in the left arm and bilateral legs.Subsequent sural nerve biopsy strongly suggested vasculitic neuropathy.Based on nerve biopsy and clinical manifestation,she was diagnosed with vasculitis and treated with immunosuppressive therapy.During treatment,sudden rectal perforation and pulmonary thromboembolism occurred,and further laboratory study suggested probable concomitant APS.Emergency Hartmann operation was performed for rectal perforation,and anti-coagulation therapy was started for APS.After few cycles of immunosuppressive therapy,tingling sensation and weakness in her hand and foot had been partially recovered and vasculitis was considered to be stationary.CONCLUSION Vasculitis can be presented with a variety of signs and symptoms,therefore,clinicians should always consider the possibility of diagnosis.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
文摘BACKGROUND Vasculitis,a systemic disorder with inflammation of blood vessel walls,can develop broad spectrum of signs and symptoms according to involvement of various organs,and therefore,early diagnosis of vasculitis is challenging.We herein describe a patient who developed a special case of systemic vasculitis with mononeuropathy multiplex,rectal perforation and antiphospholipid syndrome(APS)presented with pulmonary embolism.CASE SUMMARY A 61-year-old woman visited hospital with complaints of myalgia and occasional fever.She was initially diagnosed as proctitis and treated with antibiotics,however,there was no improvement.In addition,she also complained right foot drop with hypesthesia,and left 2^(nd) and 3^(rd) finger tingling sensation.She underwent nerve conduction study for evaluation,and it revealed sensorimotor polyneuropathy in the left arm and bilateral legs.Subsequent sural nerve biopsy strongly suggested vasculitic neuropathy.Based on nerve biopsy and clinical manifestation,she was diagnosed with vasculitis and treated with immunosuppressive therapy.During treatment,sudden rectal perforation and pulmonary thromboembolism occurred,and further laboratory study suggested probable concomitant APS.Emergency Hartmann operation was performed for rectal perforation,and anti-coagulation therapy was started for APS.After few cycles of immunosuppressive therapy,tingling sensation and weakness in her hand and foot had been partially recovered and vasculitis was considered to be stationary.CONCLUSION Vasculitis can be presented with a variety of signs and symptoms,therefore,clinicians should always consider the possibility of diagnosis.
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.
