• Journal of Medicine and Life Science
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• 제21권1호
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• pp.15-19
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• 2024

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic necrotizing vasculitis that predominantly affects small vessels of the body. The two most common ANCAs are myeloperoxidase ANCA and proteinase 3 ANCA. Neurological manifestations are frequent in patients with AAV, including peripheral neuropathy, meningitis, and stroke. AAV-associated ischemic stroke usually affects small vessels supplying the white matter or brainstem. This case report details the presentation and treatment course of a 70-year-old man with rapidly progressive multiple intracranial large artery involvement attributed to myeloperoxidase ANCA-associated vasculitis. Despite treatment with high-dose steroids and a rituximab infusion, the patient developed new speech difficulties and respiratory distress, and brain imaging confirmed new stroke lesions with progressive multiple intracranial large cerebral artery involvement. The patient died from SARS-CoV-2 infection 4 months after the diagnosis. This case emphasized the rare presentation of rapidly progressive large vessel involvement in a patient with myeloperoxidase ANCA-associated vasculitis despite active immunotherapy.

• Tuberculosis and Respiratory Diseases
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• 제39권1호
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• pp.89-94
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• 1992

저자들은 전신성홍반성낭창에서 신장염에 이어 급성 폐렴이 발생하여 이의 악화로 사망한 환자를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• 대한기관식도과학회:학술대회논문집
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• 대한기관식도과학회 1981년도 제15차 학술대회연제순서 및 초록
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• pp.13.5-14
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• 1981

Wegener써 육아종증은 1939년 Wegener가 \circled1 상하호흡기의 혈관염 및 괴사성 육아종, \circled2 전신적인 혈관염, \circled3 국한된 괴사성 사구체신염을 특징으로하는 질병을 Rhinogenic Granulomatosis라고 명명한데서 유래되었다. 초기에 이 질환은 몇달내에 사망하는 것으로 알고 있다. 그러나 최근 이 질환의 한정된 형(limited form)은 전형적인 형(classic form)에 비해 일반적으로 양성인 경과를 한다고 인지 되였다. 병리학적으로 이 질환은 한번의 조직생검으로 진단하기 힘들며 간혹 부검에서 확진되는 경우도 많다고 한다. 본교실에서는 최근 심한 시력장애, 청력장애를 일으킨 Wegener씨 육아종증 1례를 경험하였기에 이에 문헌고찰과 더불어 보고하는 바이다.

• Childhood Kidney Diseases
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• 제16권2호
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• pp.132-137
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• 2012

Henoch-Sch$\ddot{o}$nlein 자반병 신염과 유사한 증상을 가지는 10세 여자에게 발생한 급격한 신기능 저하를 동반하지 않은 현미경적 다발혈관염 1례를 경험하였기에 보고하는 바이다. 이 혈관염의 경우. 진단 시점 또는 치료 시작할 때의 신장기능 저하가 심할수록 병의 예후가 나쁘므로[20], 사구체 여과율이 떨어지기 전에 빠른 진단과 치료가 필요하다. Henoch-Sch$\ddot{o}$nlein 자반병이 의심될 경우 단백뇨와 혈뇨가 지속되면, 다른 혈관염과의 감별을 위해 ANCA, 신장 조직검사가 도움을 줄 것으로 생각된다.

• Tuberculosis and Respiratory Diseases
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• 제84권4호
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• pp.255-262
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• 2021

Microscopic polyangiitis (MPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated necrotizing vasculitis, which mainly affects small vessels in various organs, especially the lungs. The two key pulmonary manifestations, interstitial lung disease (ILD) and diffuse alveolar hemorrhage (DAH), increase the morbidity and death rate of patients with MPA. ILD is more common in MPA than in other ANCA-associated vasculitis subsets and is primarily associated with myeloperoxidase-ANCA. Unlike alveolar hemorrhage due to pulmonary capillaritis, ILD can initially manifest as isolated pulmonary fibrosis. Of note, its most frequent radiographic pattern is the usual interstitial pneumonia pattern, similar to the characteristic pattern seen in idiopathic pulmonary fibrosis. In this review we present the pathogenesis, clinical manifestations, and radiographic and histopathologic features of ILD and DAH in MPA. We also briefly summarize the outcome and therapeutic options for the two conditions.

• Investigative Magnetic Resonance Imaging
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• 제25권1호
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• pp.53-58
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• 2021

Polyarteritis nodosa (PAN) is a systemic vasculitis involving small- and medium-sized arteries, which presents with necrotizing inflammation. PAN occurs as a systemic disease or as a limited form confined to a single organ. Few cases have been reported with single organ involvement, and even fewer have been reported with skeletal muscle involvement. Herein, we report the ultrasonography and magnetic resonance imaging findings in a rare case of PAN with limited muscle involvement in a 66-year-old man.

• Journal of Yeungnam Medical Science
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• 제30권1호
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• pp.17-20
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• 2013

Wegener's granulomatosis is a very rare systemic vasculitis characterized by necrotizing granulomatosis. The detection of antineutrophil cytoplasm antibody (ANCA) is a valuable finding in diagnosing Wegener's granulomatosis because ANCA is positive in approximately 90 percent of patients with active, generalized Wegener's granulomatosis. But ANCA is not necessarily positive to make a diagnosis. A 59-year-old man was transferred to our hospital. He was diagnosed with lung abscess and treated with antibiotics at previous hospital. Initially, the ANCA was negative in immunofluorescence assay but we suspected Wegener's granulomatosis because of systemic inflammatory symptoms. Clinical symptoms deteriorated rapidly so we did bronchoscopic biopsy early. Wegener's granulomatosis was diagnosed according to pathologic finding that reported necrotizing granulomatous inflammation associated with vasculitis. Thus we treated with steroid then clinical symptoms and laboratory findings were improved.

• Journal of Yeungnam Medical Science
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• 제26권2호
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• pp.130-136
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• 2009

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that typically affects the medium-sized muscular arteries, with occasional involvement of the small muscular arteries. As with other vasculitides, PAN can affect any organ system, including the cardiovascular, gastrointestinal and central nervous systems. The prognosis for patients with untreated PAN is relatively poor, with five-year survival rates of approximately 13 percent. The outcome has improved with proper therapy to approximately 80 percent survival at five years. We report here on a case of a 46 year old man with polyarteritis nodosa and who suffered from pulmonary tuberculosis.

• 대한수의학회지
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• 제34권3호
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• pp.569-581
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• 1994

This study was carried out to investigate the pathogenesis of canine herpesvirus(CHV) infection in dogs. The 17 puppies, one day old, delivered from CHV seronegative 3 dams were divided into two groups. The 13 puppies were inoculated intranasally with 1ml of CHV-KK inoculum($5{\times}10^{5.6}TCID_{50}/ml$) and 4 puppies were served as control. And then the puppies were sacrificed at 2, 4, 6 and 7 days after the treatment, and collected blood, nasal mucosa, trigeminal nerve, trigeminal ganglion, bone marrow, eye, brain and other major organs. These organs were examined histopathologically and electron microscopically. The platelets of puppies infected with CHV were dramatically decreased because of the damages of vascular endothelial cells. Histopathologically, necrotizing vasculitis and neuritis were proceeded the generalized focal necrosis of all organs. Necrotic changes in trigeminal ganglion, trigeminal nerve and ventroposteriomedial nucleus of thalamus were observed in 4 puppies infected with CHV. Herpesviral particles, various forms of maturation, were observed in endothelial cells of the alveolar capillary and hepatic sinusoid with electron microscopy. These results suggest that the generalized focal necrcsis of all organs including brain and eyes in canine herpesvirus infection were resulted from generalized vasculitis, and also the hemonecrotizing meningoencephalitis was related to the necrosis of trigeminal nerve pathway.

• Clinical and Experimental Pediatrics
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• 제46권2호
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• pp.207-209
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• 2003

가와사끼병은 주로 심혈관계를 침범하는 전신적인 염증 질환이다. 8세 이상의 나이가 많은 소아에서 가와사끼병은 발생률이 낮고 비정형적인 다양한 임상양상을 보이기 때문에 진단이 늦어질 수 있다. 저자들은 10세 남아에서 급성 신부전과 근염을 비롯하여 여러 가지 비정형적인 증상을 보임으로써 진단이 늦어진 가와사끼병을 경험하였기에 보고하는 바이다.