• Tuberculosis and Respiratory Diseases
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• 제53권2호
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• pp.202-208
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• 2002

A diffuse alveolar hemorrhage is a rare manifestation in microscopic polyangiitis. Recently we experienced a case of diffuse alveolar hemorrhage associated with microscopic polyangiitis, which was diagnosed with the typical clinical manifestations, ANCA and a renal biopsy. A 71 year old female was admitted complaining of coughing and dyspnea. A chest X-ray, HRCT and BAL revealed a diffuse alveolar hemorrhage. A diffuse alveolar hemorrhage was noted during a bronchoscopy. She also had proteinuria, microscopic hematuria and mild azotemia. The renal biopsy showed necrotic glomerulonephritis without immune complex deposits or granuloma. Under the diagnosis of microscopic polyangiitis, she was treated with steroid pulse therapy, and prednisolone with cyclophosphamide subsequently. She showed marked improvement in the clinical manifestations.

• Tuberculosis and Respiratory Diseases
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• 제52권5호
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• pp.550-556
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• 2002

Microscopic polyangiitis is a systemic small-vessel vasculitis that is associated primarily with necrotizing glomerulonephritis and pulmonary capillaritis. A recurrent and diffuse alveolar hemorrhage due to pulmonary capillaritis is the main clinical manifestation of lung involvement. Recently, and interstitial lung disease that mimics idiopathic pulmonary fibrosis was reported to be rarely associated with microscopic polyangiitis. Here we report two patients with microscopic polyangiitis who showed a honeycomb lung at the time of the initial diagnosis with a brief review of relevant literature.

• Tuberculosis and Respiratory Diseases
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• 제72권2호
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• pp.197-202
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• 2012

Microscopic polyangiitis is a necrotizing vasculitis, characterized by inflammation of small vessels (capillaries, venules, and arterioles) with few or no immune deposits. The kidneys are the most commonly affected organs and are involved in 90% of patients, whereas pulmonary involvement occurs in a minority of cases (10% to 30%). In cases of lung disease, diffuse alveolar hemorrhage with pulmonary capillaritis is the most common manifestation. Microscopic polyangiitis is strongly associated with antineutrophil cytoplasmic autoantibody, which is a useful diagnostic serological marker. We report a case of microscopic polyangiitis presented as pleural effusion in a 67-year-old female. Pleural effusions have been reported in some cases previously, but the number of cases were small and their characteristics have not been well described. This report describes characteristic findings of pleural fluid and its histological features in a case of microscopic polyangiitis.

• Tuberculosis and Respiratory Diseases
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• 제55권6호
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• pp.623-630
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• 2003

저자들은 급성신부전과 미만성 폐포출혈이 있으면서 중추신경병증이 동반된 현미경적 다발성 혈관염 환자 l예와 특발성 괴사 사구체신염 진단 후 스테로이드 감량 도중 폐침윤이 동반된 전신적인 현미경적 다발성 혈관염의 형태로 재발한 환자 l예를 경험하였기에 문헌 고찰과 함께 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.

• Journal of Yeungnam Medical Science
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• 제21권1호
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• pp.101-107
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• 2004

Diffuse alveolar hemorrhage is a rare but serious and frequently life-threatening complication of a variety of conditions. The first goal in the management of patients with diffuse alveolar hemorrhage is to achieve or preserve stability of the respiratory status. Subsequently, the differential diagnosis is aimed at the identification of a remediable cause of the alveolar hemorrhage. The most common causes of diffuse alveolar hemorrhage with glomerulonephritis are microscopic polyangiitis and Wegener's granulomatosis, followed by Goodpasture syndrome and systemic lupus erythematosus. Microscopic polyangiitis (MPA) is a distinct systemic small vessle vasculitis affecting small sized vessels with few or no immune deposits and with no granulomatosus inflammation. The disease may involve multiple organs such as kidney, lung, skin, joint, muscle, gastrointestinal tract, eye, and nervous system. MPA is strongly associated with antineutrophil cytoplasmic autoantibody (ANCA) that is a useful serological diagnostic marker for the most common form of necrotizing vasculitis. Our report concerns a case of microscopic polyangiitis with diffuse alveolar hemorrhage in a 54-year-old man. He was admitted to our hospital due to dyspnea upon exertion and recurrent hemoptysis. Laboratory findings showed hematuria, proteinuria and deterioration of renal function. In the chest CT scan, diffuse ground glass appearance was seen in both lower lungs. A lung biopsy revealed small vessel vasculitis with intraalveolar hemorrhage and showed a positive reaction to against perinuclear ANCA. The patient was treated with prednisolone and cyclophosphamide. Chest infiltration decreased and hemoptysis and hypoxia improved. He is still being followed up in our hospital with a low dose of prednisolone.

• 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.

• Tuberculosis and Respiratory Diseases
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• 제70권3호
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• pp.257-260
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• 2011

A 65-year-old woman was admitted due to poor oral intake and a dry cough over the previous 3 months. The physical examination was remarkable for bibasilar crackles, and plain chest radiography showed reticulation in both lower lung fields. A pulmonary function test demonstrated a restrictive pattern with a reduced diffusing capacity of the lung for carbon monoxide. High resolution computed tomography showed reticulation and honey-combing in both peripheral lung zones, which was consistent with usual interstitial pneumonia pattern. Her skin showed livedo reticularis. The erythrocyte sedimentation rate and C-reactive protein level were elevated, and hematuria was noted on urinary analysis. A serologic test for auto-antibodies showed seropositivity for Myeloperoxidase-Anti-neutrophil cytoplasmic antibody (MPO-ANCA). A kidney biopsy was performed and showed focal segmental glomerulosclerosis. She was diagnosed as having pulmonary fibrosis with microscopic polyangiitis (MPA) and treated with high dose steroids. Here we report a case of pulmonary fibrosis coexistent with microscopic polyangiitis.

• Childhood Kidney Diseases
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• 제12권1호
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• pp.99-104
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• 2008

현미경적 다발성 동맥염은 폐출혈과 급속 진행성 사구체심염을 특징으로 하는 전신성 혈관염의 일종으로 소아에서는 매우 드문 질환이다. 저자들은 폐출혈과 급성 신부전을 동반한 7세 여아에서 신장조직검사와 p-ANCA 검사로 현미경적 다발성 동백염으로 진단 후 5년 뒤 2차 신장조직검사와 경구 ACE inhibitor, angiotensin II receptor blocker와 저용량의 cyclophosphamide를 투여 받은 환아를 7년간 추적관찰하였다. 발병당시에는 BUN 117 mg/dL, Cr 2.3 mg/dL이었으나, 퇴원시 BUN 20.8 mg/dL, Cr 1.6 mg/dL이었고, 최근 검사에서는 BUN 51.7 mg/dL, Cr 3.2 mg/dL으로 만성 신병증 소견을 보이고 있으며, 외래 추적 관찰 지속 중에 있다. 이에 문헌 고찰과 함께 증례 보고하는 바이다.

• Journal of Chest Surgery
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• 제44권6호
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• pp.448-451
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• 2011

Microscopic polyangiitis (MPA) is a necrotizing vasculitis involving the small vessels without granulomatous inflammation. Most MPA initially presents with renal involvement without pulmonary involvement. Isolated and initially presenting alveolar hemorrhage is very rare. The patient was a 39-year-old female with a progressive cough, dyspnea, and blood-tinged sputum for the previous 5 days. We determined that her condition was MPA though VATS lung biopsy and renal biopsy. After 2 months of steroid therapy, the chest lesions had improved. We report here a rare case of MPA with isolated and initial involvement of the lung with a review of the literature.