• Title/Summary/Keyword: Systemic vasculitis

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A Case of Cerebral Vasculitis in Henoch-Shönlein Purpura (알레르기성 자반증에 병발한 대뇌 혈관염 1례)

  • Lee, Soo Yeon;Choung, Ju Mi;Hwang, Dong Jin;Eun, So Hee;Hwang, Pyoung Han
    • Clinical and Experimental Pediatrics
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    • v.45 no.12
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    • pp.1601-1605
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    • 2002
  • Henoch-Sh${\ddot{o}}$nlein Purpura(HSP) is an immuologically mediated systemic vasculitis of small blood vessels affecting predominantly the skin, gastrointestinal tract, joints, and kidneys. Clinical neurological manifestations such as headaches, behavioral changes, mental changes, seizures, and visual loss are described, but neurological complication are rare during the course of HSP. We experienced a case of an 8 year-old male with HSP who presented with seizures. Magnetic resonance imaging(MRI) showed multiple high signal intensity in both cortical and subcortical areas of frontal and parieto-occipital lobes and magnetic resonance(MR) angiogram showed stenosis of cerebral arteries, compatible with MRI and MR angiogram findings of cerebral vasculitis. We report this case with related literature.

A Case of Fatal Acute Lupus Pneumonitis Defined by Necropsy (병리조직학적으로 확인된 급성루프스성 폐렴 1예)

  • Yoon, Ki-Heon;Yoo, Jee-Hong;Kang, Hong-Mo
    • Tuberculosis and Respiratory Diseases
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    • v.39 no.1
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    • pp.89-94
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    • 1992
  • A 16 years old girl with systemic lupus erythematosus had a high fever for 20 days. Skin and renal biopsy showed diffuse granular deposits (IgG, IgM, $C_3$, $C_{1q}$ at dermo-epideral junction and IgG, IgA, IgM, $C_3$, $C_{1q}$, fibrinogen in the renal mesangium and segmentally along the capillary walls) which were compatable with systemic lupus erythematosus. The chest X-ray revealed patchy mottled densities in whole lung field when she complained more dyspnea at 9th hospital days. Even with the parenteral administration of broad-spectrum antibiotics, the symptoms of high fever, cough, tachydyspnea and hypoxia were continued. At 24th hospital day, the clinical course was rapidly deteriorated after sudden loss of consciousness with focal seizure which suggested CNS involvement during hydrocortisone administration for 10 days. She died of respiratory failure despite the mechanical ventilatory support with PEEP. The limited necropsy showed interstitial pneumonia, alveolar hemorrhage and occlusive necrotizing vasculitis of acute lupus pneumonitis.

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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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Understanding the importance of cerebrovascular involvement in Kawasaki disease

  • Yeom, Jung Sook;Cho, Jae Young;Woo, Hyang-Ok
    • Clinical and Experimental Pediatrics
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    • v.62 no.9
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    • pp.334-339
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    • 2019
  • Kawasaki disease (KD) is a systemic vasculitis in infants and young children. However, its natural history has not been fully elucidated because the first case was reported in the late 1960s and patients who have recovered are just now entering middle age. Nevertheless, much evidence has raised concerns regarding the subclinical vascular changes that occur in post-KD patients. KD research has focused on coronary artery aneurysms because they are directly associated with fatality. However, aneurysms have been reported in other extracardiac muscular arteries and their fate seems to resemble that of coronary artery aneurysms. Arterial strokes in KD cases are rarely reported. Asymptomatic ischemic lesions were observed in a prospective study of brain vascular lesions in KD patients with coronary artery aneurysms. The findings of a study of single-photon emission computed tomography suggested that asymptomatic cerebral vasculitis is more common than we believed. Some authors assumed that the need to consider the possibility of brain vascular lesions in severe cases of KD regardless of presence or absence of neurological symptoms. These findings suggest that KD is related with cerebrovascular lesions in children and young adults. Considering the fatal consequences of cerebral vascular involvement in KD patients, increased attention is required. Here we review our understanding of brain vascular involvement in KD.

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 Tuberculous Meningoencephalitis Presenting as Prominent Psychotic Features (현저한 정신증상으로 발현된 결핵성 뇌수막뇌염 1예)

  • Yu, Hyun-Jung;Oh, Jee-Young;Kim, Jee-Hyun;Kim, Jung-Eun;Park, Kee-Duk
    • Tuberculosis and Respiratory Diseases
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    • v.51 no.6
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    • pp.603-608
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    • 2001
  • Intracranial vasculitis is a rare and disastrous complication of tuberculous meningitis if not treated properly. Focal neurologic deficits according to the vessels involved are common manifestation. Here, we report a 29-year-old man who suffered abrupt, bizarre behavioral changes caused by vasculitis complicating tuberculous meningoencephalitis. The diagnosis of tuberculous meningitis is based upon both the CSF findings and a chest X-ray. His systemic symptoms disappeared by after being administered antituberculous medication but various psychotic features such as hypersomnia, hyperphagia and aggressive behavior continued. A brain MRI showed multiple small parenchymal tuberculous nodules, and the brain MR angiography revealed a narrowing of the proximal middle cerebral arteries and a reduced visualization of the cerebral vessels, suggesting widespread vasculitis. Intravenous dexamethasone successfully ameliorated his behavioral changes. In addition both the follow up brain MRI and angiography showed a normalization of the previous findings.

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Predictors and management of intravenous immunoglobulin-resistant Kawasaki disease

  • Song, Min Seob
    • Clinical and Experimental Pediatrics
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    • v.62 no.4
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    • pp.119-123
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    • 2019
  • Kawasaki disease (KD) is a systemic vasculitis that mainly affects younger children. Intravenous immunoglobulin (IVIG) resistant cases are at increasing risk for coronary artery complications. The strategy on prediction of potential nonresponders and treatment of IVIG-resistant patients is now controversial. In this review the definition and predictors of IVIG-resistant KD and current evidence to guide management are discussed.

A Case of ANCA-Negative Generalized Wegener's Granulomatosis (ANCA 음성인 전신성 베게너육아종증 1예)

  • Kim, Seung Kyu;Kwon, Yong-Jin;Park, Heae Surng;Rhee, Kwang Won;Ha, Ji Yoon;Ko, Hee Sung;Kim, Ki Hyun;Byun, Min Kwang
    • Journal of Yeungnam Medical Science
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    • v.30 no.1
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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.

A Case of Polyarteritis Nodosa Associated with Pulmonary Tuberculosis (폐결핵에 동반된 결절성다발성동맥염 1례)

  • Son, Chang-Woo;Cho, Jeong-Hwan;Song, In-Wook;Park, Jung-Eun;Shin, Kyeong-Cheol;Chung, Jin-Hong;Lee, Kwan-Ho
    • Journal of Yeungnam Medical Science
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    • v.26 no.2
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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.

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A Case of Wegener's Granulomatosis (Wegener씨 육아종증 1례)

  • 박성준;권평중;김중환
    • Proceedings of the KOR-BRONCHOESO Conference
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    • 1981.05a
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    • pp.13.5-14
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    • 1981
  • Wegener's granulomatosis is characterized by 3 criteria: 1. Necrotizing granulomas with vasculitis of upper and lower respiratory tracts 2. A systemic vasculitis 3. Focal necrotizing glomerulitis. This disease is one of the nonhealing disease in the otolaryngologic and ophthalmologic fields. A 48years old Korean male patient was seen with the complaints of nasal discharge, foul odor and frequent nasal bleeding. The patient was admitted after biopsy of the nasal cavity which diagnosed tuberculous granuloma, for biopsy of the maxillary sinus. After biopsy by Caldwell-Luc's approach this patient was complained with severe headache, visual impairment and cough. And so this patient was readmitted for further evaluation. Generally, the diagnosis was made after autopsy sometimes several years later after reevaluation of the case. Tuberculous granuloma was the pathological diagnosis on the basis of resected material in various cases. The correct diagnosis was made at autopsy occasionally. It is our intention to present this case with. literature review.

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