• Title/Summary/Keyword: Necrotizing vasculitis

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A Case of Churg-Strauss Syndrome with Multiple Tracheobronchial Mucosal Lesions (기관과 기관지내 다발성 점막 병변을 동반한 Churg-Strauss 증후군 1예)

  • Boo, Sun-Jin;Lee, Kwangha;Ra, Seung Won;Jin, Young-Joo;Park, Gyung-Min;Hong, Sang-Bum
    • Tuberculosis and Respiratory Diseases
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    • v.65 no.5
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    • pp.405-409
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    • 2008
  • Churg-Strauss syndrome is a rare form of systemic necrotizing vasculitis that occurs exclusively in patients with asthma, and is associated with blood and tissue eosinophilia. The classic pathology findings in the lung include a combination of eosinophilic pneumonia, granulomatous inflammation and necrotizing vasculitis. However, there are few reports of tracheobronchial mucosal lesions in Churg-Strauss syndrome. We report a case of Churg-Strauss syndrome with multiple tracheobronchial mucosal lesions in a 33-year-old man with a history of bronchial asthma and allergic rhinitis. He had been diagnosed with community acquired pneumonia at another hospital and was treated with antibiotics. However, the chest radiographic findings were aggravated and showed multifocal consolidations in the whole lung fields. He was transferred to the Asan Medical Center. Fiberoptic bronchoscopy revealed multiple nodular mucosal lesions of the trachea and bronchi. The histopathology of the mucosal lesions revealed necrotizing bronchial inflammation with eosinophilic infiltration. Video Assisted Thoracic Surgery was performed. The wedge resected lung tissue revealed chronic eosinophilic pneumonia that was consistent with Churg-Strauss syndrome. Methylprednisolone (1 mg/kg q 8 hr) was prescribed and his symptoms resolved gradually. The chest radiographic findings improved significantly, and a follow-up fiberoptic bronchoscopy performed eight days later showed that the tracheobronchial mucosal lesions had resolved. The patient was prescribed oral prednisolone for 20 months after discharge. Currently, the patient is not taking steroids and is being followed up.

A Case of Microscopic Polyangiitis with Diffuse Alveolar Hemorrhage (폐출혈을 동반한 현미경적 다발혈관염 1예)

  • Lee, Sang-Jin;Lee, Jae-Woung;Kim, Hye-Jin;Shin, Kyeong-Cheol;Chung, Jin-Hong;Lee, Kwan-Ho;Park, Hye-Jung
    • Journal of Yeungnam Medical Science
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    • v.21 no.1
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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.

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A Case of Nephrotic Syndrome in Behcet Syndrome (신 증후군을 동반한 베체트 증후군(Behcet syndrome) 1례)

  • Juhng Woo-Suk;Kim Hye-Young;Lee Dae-Yeol
    • Childhood Kidney Diseases
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    • v.2 no.1
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    • pp.73-76
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    • 1998
  • Behcet syndrome is a multisystem disease complex of unknown cause. It is usually manifested by aphthous oral and genital ulcers, uveitis and skin eruption. Less frequently, CNS involvement, colitis, large vessel vasculitis, and myocarditis occur. Recently, several studies have reported renal manifestations of amyloidosis and focal necrotizing glomerulonephritis in Behcet syndrome. We describe a patient with Behcet syndrome who experienced nephrotic syndrome with focal effacement of epithelial foot process in glomeruli. A brief review of literature ensues.

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Aspergillosis in a Blue-fronted Amazon (Amazona aestiva) (아마존앵무에서 Aspergillus 감염증)

  • Kwon Hyo-jung;Park Mi-sun;Kim Dae-yong;Hwang Cheol-yong;Shin Nam-shik
    • Journal of Veterinary Clinics
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    • v.21 no.4
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    • pp.413-415
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    • 2004
  • A male Blue-fronted amazon parrot suddenly died after having a history of depression, anorexia and emaciation. At necropsy, numerous well-demarcated yellow to white firm nodules were scattered throughout the left caudal lung lobes and adjacent air sac. Histologically, the wall of air sac was significantly thickened due to necrosis and infiltration of large numbers of fungi and degenerated heterophils. Multifocal necrotizing granulomatous lesions with numerous fungal hypae as well as vasculitis and thrombosis were found in tl1e lung. The fungi had conidiophore, vesicle, phialides and conidia which were characteristic of Aspergillus sp. This is tl1e first report of mycotic air sacculitis and pneumonitis caused by Aspergillus in a parrot in Korea.

Effect of lower extremity resistance exercise on gait performance in a patient with systemic lupus erythematosus with cerebral infarction and lower extremity vasculitis: a case study

  • Oh, Yongseop;Woo, Youngkeun
    • Physical Therapy Rehabilitation Science
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    • v.5 no.2
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    • pp.106-112
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    • 2016
  • Objective: This study was conducted to investigate the effects of resistant exercise on the gait performance of a patient with systemic lupus erythematosus (SLE) patient. Design: A case study. Methods: A 30-year-old male adult who had been diagnosed with systemic lupus erythmatosus (SLE) in April 2013, right middle cerebral artery infarction, and with left hemiplegia agreed to participate in this case study. Patient was unable to walk due to being affected with adynamia. Due to developing necrotizing vasculitis on the left lower extremity, patient underwent a myotomy on the left thigh. The patient was trained with a progressive resistant exercise program for 8 weeks. An intensity of 15 RM was used for the resistant exercises and the resistance level was increased progressively in order to improve the muscle power of the patient. Methods used to increase resistance included changing positions, providing mechanical resistance instead of manual resistance, transitioning from open kinetic chain to closed kinetic chain exercises, and changing the colors of the theraband to those with increase level of resistance. Outcome measures included the 5-repetition sit-to- stand test (5RSST), Timed Up & Go (TUG), and 10-meter walk test (10MWT). In addition, the GAITRite was used to assess the spatio-temporal gait variables, including gait speed, cadence, stride length of the left side, and double limb support pre and post-intervention. Results: The patient was able to perform sit-to- stand after two weeks of performing the resistant exercises. The patient was able to walk after 4 weeks, and the patient's overall gait performance had improved after 8 weeks. All of the variables had improved after each week. Conclusions: The results of this case study may be used to enhance future efforts to objectively evaluate resistant exercises during gait performance in persons affected by SLE.

A Case of Churg-Strauss Syndrome with Interstinal Perforation (장천공이 동반된 Churg-Strauss 증후군 1예)

  • Park, Jung Hoon;Jung, Yun Seok;Kim, Yang Ki;Lee, Young Mok;Hwang, Jung Hwa;Kim, Ki-Up;Kim, Dong Won;Uh, Soo-Taek;Kim, Jae Jun
    • Tuberculosis and Respiratory Diseases
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    • v.66 no.5
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    • pp.374-379
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    • 2009
  • Churg-Strauss syndrome (CSS) or allergic granulomatous angiitis is a rare syndrome that is characterized by hypereosinophilic systemic necrotizing vasculitis affecting small- to medium-sized arteries and veins. In general, it occurs in individuals with pre-existing allergic asthma. When CSS appears in patients, it has the following characteristics: eosinophilia of more than 10% in peripheral blood, paranasal sinusitis, pulmonary infiltrates, histological proof of vasculitis with extravascular eosinophils, and mononeuritis multiplex or polyneuropathy. Therapeutic trials dedicated to Churg-Strauss syndrome have been limited due to the rarity of this disorder and the difficulty in making a histological diagnosis. Proper treatment of patients with CSS is not widely known. In this case study, we report on our experience with an unusual patient case, characterized by purpura and a perforation of the small intestine after inadequate steroid therapy.

A Case of Churg-Strauss Syndrome with Diffuse Alveolar Hemorrhage Presenting as Acute Acalculous Cholecystitis (미만성 폐포 출혈을 동반한 급성 담낭염으로 발현한 Churg-Strauss 증후군 1예)

  • Kim, Ji Eun;Kim, Ki Uk;Park, Hye-Kyung;Jeon, Doo Soo;Kim, Yun Sung;Lee, Min Ki;Park, Soon Kew
    • Tuberculosis and Respiratory Diseases
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    • v.66 no.3
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    • pp.225-229
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    • 2009
  • Churg-Strauss syndrome (CSS) is a disorder that is characterized by asthma, hypereosinophilia and systemic vasculitis affecting a number of organs. The manifestations of acute cholecystitis and diffuse alveolar hemorrhage are rarely reported in CSS. A 22-year-old woman with bronchial asthma visited our hospital complaining of right upper quadrant pain with a sudden onset. The abdominal computed tomography (CT) scan revealed gall bladder edema consistent with acute cholecystitis. On the initial evaluation, marked hypereosinophilia was observed in the peripheral blood smear. The nerve conduction velocity measurements and a skin biopsy performed to confirm the organ involvement of disease indicated typical mononeuritis multiplex and necrotizing vasculitis, respectively, which was complicated with CSS. On the 12th hospital day, ground glass opacity and consolidations were newly developed on both lung fields. The bronchoalveolar lavage (BAL) fluid showed increasing bloody return in sequential aliquots that were characteristic of a diffuse alveolar hemorrhage. We report a case of CSS with acute cholecystitis and diffuse alveolar hemorrhage.

An overlap syndrome of Churg-Strauss syndrome and rheumatoid arthritis

  • Bae, Seung Il;Jang, Jong Geol;Kim, Hun Tae;Ahn, Hee Yun;Kim, Min Jung;Kim, Hyun Je;Lee, Choong Ki;Hong, Young Hoon
    • Journal of Yeungnam Medical Science
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    • v.32 no.2
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    • pp.127-131
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    • 2015
  • Churg-Strauss syndrome (CSS) is a necrotizing vasculitis with extra-, peri-vascular eosinophilic infiltration. Chronic symmetric polyarthritis with the presence of rheumatoid factor (RF) and anti-cyclic citrullinated peptide antibody are the mainstay of rheumatoid arthritis (RA) diagnosis. Mononeuritis multiplex is a peripheral neuropathy involving more than 2 separate nerve areas. A 62-year-old male patient was referred for left foot drop and polyarthritis of both hands and feet for 4 months. During evaluation, mononeuritis multiplex was detected on nerve conduction study and electromyography tests: vasculitis with neutrophil, eosinophil, and lymphocyte infiltration on peroneal nerve biopsy. A positive response to methacholin and bronchodilator was observed on the pulmonary function test. Radiologic tests showed peri-articular soft tissue swelling and osteopenia on both hand and foot. Marked peripheral eosinophilia, high RF, and positive perinuclear anti-neutrophil cytoplasmic antibody were detected on blood tests. Here, we report on a patient with overlap syndrome of CSS and RA with review of the relevant literature, from which a few references to overlap syndrome of CSS and RA were available.

Two Cases of Microscopic Polyangiitis (현미경적 다발성 혈관염 2예)

  • Song, Kyoung Eun;Im, Seung Guan;Jo, Sook Kyoung;Jung, Youn Mu;Choi, Joon Hyuck;Sheen, Seung Soo;Oh, Yoon Jung;Park, Kwang Joo;Hwang, Sung Chul;Yim, Hyun Ee
    • Tuberculosis and Respiratory Diseases
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    • v.55 no.6
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    • pp.623-630
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    • 2003
  • Microscopic polyangiitis is a systemic small-vessel vasculitis that is primarily associated with necrotizing glomerulonephritis and pulmonary capillaritis. Lung involvement is characterized by a diffuse alveolar hemorrhage. However, rarely central nervous system involvement has been reported to be occurred with the microscopic polyangiitis. Relapse of microscopic polyangiitis are reported to be more frequent than those of polyarteritis nodosa, often after a reduction or discontinuation of the therapy. We would like to report two patients with microscopic polyangiitis. One presented with clinical manifestations of both lung and central nervous system involvements and the other was a case of recurrence during steroid tapering following the steroid pulse therapy.

Osteonecrosis of Maxilla in Wegener's Granulomatosis: Case Report (상악골 괴사를 동반한 베게너 육아종증: 증례보고)

  • Kim, Il-Kyu;Chang, Jae-Won;Ju, Sang-Hyun;Pyeon, Young-Hoon;Jung, Bum-Sang;Han, Ji-Young
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.34 no.3
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    • pp.220-225
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    • 2012
  • Wegener's granulomatosis (WG) is an autoimmune disease of unknown etiology characterized by the triad of necrotizing granulomatous lesion in the upper and lower respiratory tracts or both, disseminated vasculitis involving both small arteries and veins, and necrotizing glomerulonephritis. The most common oral lesions associated with WG are ulceration and strawberry gingivitis. A 47-years old man in medical care associated WG was consulted our Division of Oral and Maxillofacial Surgery for the chief complaint of toothaches. Pre-operative panorama showed the alveolar radiolucency and the loss of lamina dura regarding the left upper teeth. An oropharyngeal magnetic resonance imaging also revealed the increased bone marrow signal intensity on the left maxilla. Under the impression of maxillary osteonecrosis due to WG, maxillary saucerization with removal of involved teeth was performed. We obtained good results and report the first case of WG in Korea, with the review of literatures regarding oral and general systemic features.