• 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.

• 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.

• 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.

• Tuberculosis and Respiratory Diseases
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• v.63 no.6
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• pp.531-536
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• 2007

Wegener's granulomatosis is a systemic vasculitis of the medium and small arteries, as well as of the venules, arterioles, and occasionally large arteries, and primarily involves the upper and lower respiratory tracts and the kidneys. Renal symptoms of Wegener's granulomatosis are indistinguishable from those of vasculitis such as Henoch-$Sch\ddot{o}nlein$ purpura and microscopic polyangiitis. This case, though initially diagnosed as Henoch-$Sch\ddot{o}nlein$ purpura, was confirmed as Wegener's granulomatosis from a lung biopsy fifteen years after the initial diagnosis. We report this case with a review of the literature.

• Tuberculosis and Respiratory Diseases
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• v.67 no.3
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• pp.234-238
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• 2009

Henoch-$Sch\ddot{o}nlein$ Purpura (HSP) is a systemic vasculitis involving small vessels with the deposition of immune complexes containing IgA and C3, which is characterized by associated skin, joint, renal, and gastrointestinal manifestations. Although there were several causes such as autoimmunity, infection, and drugs, it also can be presented as a form of vasculitidies associated with malignancies. We report a case of HSP developed during treatment for non-small cell lung cancer in a 66-year-old man. Multiple purpuric skin lesions occurred in both legs after the first cycle of chemotherapy, which was diagnosed as HSP by clinical and pathologic examinations. Due to the itching sensation, topical steroid was applied and the patient was improved 3 weeks later without a scheduled change in chemotherapy.

• Tuberculosis and Respiratory Diseases
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• v.61 no.1
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• pp.65-69
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• 2006

Behcet's disease is a systemic vasculitis of an unknown etiology involving the arteries and veins of all sizes. There are reports showing that a pulmonary artery aneurysm or thromboembolism and superior vena cava thrombosis are present in 5-10% of patients with Behcet's disease and that lung parenchymal lesions are mainly airway consolidations resulting from hemorrhage or infarction. We encountered a patient with increasing pulmonary cavitary changes and localized aspergilloma. The patient was a 43-year-old man diagnosed with Behcet's disease with a history of recurrent oro-genital ulceration and uveitis, and who was administered methotrexate, colchicines, prednisolone. During the follow up he developed progressive dyspnea upon exertion and finger clubbing. Therefore further evaluations were performed. Chest computed tomography showed more advanced consolidations and cavitations than the previous film with the previously known aspergilloma still observable. An open lung biopsy was carried out to determine the presence of malignant changes, which revealed nonspecific vasculitis. Azathioprine was added resultion in an improvement of symptoms.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.213-218
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• 2001

Microscopic Polyangiitis(MPA) belongs to a spectrum of systemic vasculits, and particularly antineutrophil cytoplasmic autoantibodies(ANCA)-associated small-vessel vasculitis which is characterized by involvement of the lung and kidney. The diagnosis of MPA is often difficult to make, and delayed because of the variability of the clinical presentation. Renal biopsies have a very important diagnostic and prognostic value in MPA. We experienced a case of microscopic polyangiits which was confirmed by renal biopsy and positive serum perinuclear ANCA, associated with alveolar hemorrhage and gastrointestinal bleeding. We began methylprednisolone pulse therapy, combined with a low dose of cyclophosphamide and plasmapheresis therapy. ACE inhibitor and Ca channel blocker were used when proteinuria and hypertention developed. On admission, the patient's lab findings showed BUN l17 mg/dL, Cr 2.3 mg/dL, while on the 60th hospital day BUN/Cr values fell to 20.8 mg/dL / 1.6 mg/dL and though proteinuria persisted, the patient's condition was tolerable and is currently under observation on an out-patient basis. The last lab values were BUN 26 mg/dL / Cr 1.6 mg/dL. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 213-18)

• Journal of Yeungnam Medical Science
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• v.34 no.1
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• pp.111-114
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• 2017

Central serous chorioretinopathy may induce poor eyesight and serous retinal detachment. However, its exact cause has not been well established thus far. It can be associated with systemic high-dose corticosteroid treatment mainly for young and middle-aged men and may spontaneously regress or recur after withdrawal from corticosteroid. After corticosteroid administration for Behcet's disease, it is necessary to identify any ocular symptoms. Behcet's disease can lead to the development of ocular complications, such as uveitis, hypopyon, retinal vasculitis, optic neuritis, angiogenesis, secondary cataract, and glaucoma. It is possible to diagnose any of these complications via optical coherence tomography and digital indocyanine green angiography. It is easy to neglect an ocular symptom that may appear after a low-dose corticosteroid treatment as an ocular complication in patients with Behcet's disease. Thus, we report on a case concerning high-dose corticosteroid treatment with a literature review.

• Journal of Chest Surgery
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• v.45 no.1
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• pp.30-34
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• 2012

Background: The long-term results of homografts used in systemic circulation are controversial. We assessed the long-term results of using a cryopreserved homograft for an aortic root or aorta and its branch replacement. Materials and Methods: From June 1995 to January 2010, 23 patients (male:female=15:8, $45.4{\pm}15.6$ years) underwent a homograft replacement in the aortic position. The surgical techniques used were aortic root replacement in 15 patients and aortic graft interposition in 8 patients. Indications for the use of a homograft were systemic vasculitis (n=15) and complicated infection (n=8). The duration of clinical follow-up was $65{\pm}58$ months. Results: Early mortality occurred in 2 patients (8.7%). Perioperative complications included atrial arrhythmia (n=3), acute renal failure (n=3), and low cardiac output syndrome (n=2). Late mortality occurred in 6 patients (26.1%). The overall survival rates at 5 and 10 years were 66.3% and 59.6%, respectively. Six patients (28.6%) suffered from homograft-related complications. Conclusion: Early results of homograft replacement in aortic position were favorable. However, close long-term follow-up is required due to the high rate of homograft-related events.

• Tuberculosis and Respiratory Diseases
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• v.53 no.2
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• pp.183-189
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• 2002

Churg-Strauss syndrome(CSS) is a systemic vascular disorder that has an unknown cause with multiorgan involvement and diverse presentations. The three main histologically distinct phases were necrotizing vasculitis, tissue eosinophilia and extravascular granulomas. A diagnosis of CSS can be made on four or more of the following six criteria : 1) asthma, 2) peripheral eosinophilia >10% on the differential leukocyte count, 3) mononeuropathy (including multiple) or polyneuropathy, 4) paranasal sinus abnormalities, 5) nonfixed pulmonary infiltrates and 6) biopsy evidence of extravascular eosinophils in the skin, the nerves, or the lungs. CSS has a good prognosis with systemic steroid therapy. The 5 year survival is approximately 70%. We experienced a 66-year-old man who presented with cough, sputum, edema and numbness in both legs. He presented with all of the 6 CSS criteria. A nerve and muscle biopsy confirmed the diagnosis. Here, we report this case with a review of the relevant literatures.