• Journal of Medicine and Life Science
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• v.21 no.1
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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.

• Archives of Plastic Surgery
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• v.33 no.1
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• pp.116-119
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• 2006

Management of chronic wound has been a great problem to many surgeons because the wound is usually associated with an underlying disease of the patient. Without accurate diagnosis and treatment of the disease, the wound can not be healed. Systemic vasculitis is a rare systemic disease which causes inflammation and obstruction of the vessels. This autoimmune disease involves multiple organs and may inflict skin wound spontaneously without traumas. It would improve or aggravate the wound in proportion to the activities of the disease. Our experience is a case of 28-year-old female who has had chronic ulcers on her right foot, especially on the great toe for 1 year. Although she had several operations of sympathectomy, debridement and artificial dermal graft, her wound was not improved. She has been diagnosed as systemic vasculitis during the evaluation for histopathology and cause of fever and pancytopenia. After medical treatments, she had the operation of adipofascial turnover flap coverage and skin graft, and the wound was improved without any complication or relapse. The diagnosis and treatment of the underlying disease should be ahead of the management of chronic wound.

• Tuberculosis and Respiratory Diseases
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• v.43 no.5
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• pp.786-791
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• 1996

Systemic sclerosis is a multisystemic disease of unknown origin charicterized by degenerative fibrotic and inflammatory changes in the skin, vessels, joints, muscles, and visceral organs. Involvement of the lung in systemic sclerosis is common, but pleural effusion is rare. Although vasculitis commonly accompanies many connective tissue disorders, it has been rarely reported in systemic sclerosis. A 43-year-old woman, with a 10-year history of Raynaud's phenomenon, was admitted due to right chest pain. Her hands showed diffuse thickening and swelling of skin. Chest X-ray showed pleural effusions and esophageal manometry showed hypotonic peristalsis and low lower esophageal sphincter tone compatible with scleroderma esophagus. Antinuclear antibodies were present (titer>1 : 160) with a speckled pattern. She was positive for rheumatoid factor, anti scl-70 and RNP antibodies, but negative for anti-Ro, La, and Sm antibodies. Histology of the pleura revealed the presence of leukocytoclastic vasculiti. After adminisrration of prednisolone 30 mg/day, her chest symptom was improved. We report a case of systemic sclerosis with pleural effusions due to leukocytoclastic vasculitis with review of the literatures.

• Journal of the Korean Society of Radiology
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• v.82 no.4
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• pp.791-807
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• 2021

Vasculitis is a systemic disease, characterized by inflammation of the vascular wall. Although rare, it is sometimes life-threatening due to diffuse pulmonary hemorrhage or acute glomerulonephritis. Besides primary vasculitis, whose cause is unknown, numerous conditions such as autoimmune diseases, drugs, infections, and tumors can cause secondary vasculitis. Vasculitis displays various non-specific symptoms, signs, and laboratory findings; hence, diagnosis of the disease requires integration of various results including clinical features, imaging findings, autoantibody tests, and pathological findings. In this review, we have discussed the clinical, radiologic, and pathological features of vasculitis. Further, we elaborated the imaging findings and differential diagnosis of typical vasculitis that frequently involves the lung and introduced a new international classification of vasculitis, the Diagnostic and Classification Criteria in Vasculitis.

• Tuberculosis and Respiratory Diseases
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• v.74 no.4
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• pp.151-162
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• 2013

Diffuse alveolar hemorrhage (DAH) is a life-threatening and medical emergency that can be caused by numerous disorders and presents with hemoptysis, anemia, and diffuse alveolar infiltrates. Early bronchoscopy with bronchoalveolar lavage is usually required to confirm the diagnosis and rule out infection. Most cases of DAH are caused by capillaritis associated with systemic autoimmune diseases such as anti-neutrophil cytoplasmic antibody-associated vasculitis, anti-glomerular basement membrane disease, and systemic lupus erythematosus, but DAH may also result from coagulation disorders, drugs, inhaled toxins, or transplantation. The diagnosis of DAH relies on clinical suspicion combined with laboratory, radiologic, and pathologic findings. Early recognition is crucial, because prompt diagnosis and treatment is necessary for survival. Corticosteroids and immunosuppressive agents remain the gold standard. In patients with DAH, biopsy of involved sites can help to identify the cause and to direct therapy. This article aims to provide a general review of the causes and clinical presentation of DAH and to recommend a diagnostic approach and a management plan for the most common causes.

• Investigative Magnetic Resonance Imaging
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• v.25 no.1
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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.

• Clinical and Experimental Pediatrics
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• v.63 no.5
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• pp.158-163
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• 2020

IgA vasculitis, formerly known as Henoch-Schönlein purpura, is a systemic IgA-mediated vasculitis of the small vessels commonly seen in children. The natural history of IgA vasculitis is generally self-limiting; however, one-third of patients experience symptom recurrence and a refractory course. This systematic review examined the use of dapsone in refractory IgA vasculitis cases. A literature search of PubMed databases retrieved 13 articles published until June 14, 2018. The most common clinical feature was a palpable rash (100% of patients), followed by joint pain (69.2%). Treatment response within 1-2 days was observed in 6 of 26 patients (23.1%) versus within 3-7 days in 17 patients (65.4%). Relapse after treatment discontinuation was reported in 17 patients (65.4%) but not in 3 patients (11.5 %). Four of the 26 patients (15.4%) reported adverse effects of dapsone including arthralgia (7.7%), rash (7.7%), and dapsone hypersensitivity syndrome (3.8%). Our findings suggest that dapsone may affect refractory IgA vasculitis. Multicenter randomized placebo-controlled trials are necessary to determine the standard dosage of dapsone at initial or tapering of treatment in IgA vasculitis patients and evaluate whether dapsone has a significant benefit versus steroids or other medications.

• Tuberculosis and Respiratory Diseases
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• v.79 no.4
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• pp.302-306
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• 2016

Systemic vasculitis involving the lung is a rare manifestation of myelodysplastic syndrome (MDS), and secondary vasculitis is considered to have poor prognosis. A 44-year-old man presented with fever and dyspnea of 1 month duration. A chest radiograph revealed bilateral multiple wedge shaped consolidations. In addition, the results of a percutaneous needle biopsy for non-resolving pneumonia were compatible with pulmonary vasculitis. Bone marrow biopsy was performed due to the persistence of unexplained anemia and the patient was diagnosed with MDS. We reported a case of secondary vasculitis presenting as non-resolving pneumonia, later diagnosed as paraneoplastic syndrome of undiagnosed MDS. The cytopenia and vasculitis improved after a short course of glucocorticoid treatment, and there was no recurrence despite the progression of underlying MDS.

• Clinical and Experimental Pediatrics
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• v.46 no.10
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• pp.1040-1043
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• 2003

Henoch-Shonlein purpura(HSP) is a systemic small-vessel vasculitis that primarily affects the skin, gastrointestinal tract, joints, and kidneys. The nervous system may be involved, less commonly than other organs. When the central nervous system(CNS) was involved, headache, changes in mental status, seizures, and focal neurologic deficits have been reported. Hypertension, uremic encephalopathy, metabolic abnomalities, electrolyte abnormalities, or cerebral vasculitis were suggested as possible causes of the neurologic manifestation. Diagnosis of vasculitic involvement of CNS is difficult. Magnetic resonance imaging of the brain is the modality of choice for the evaluation of the CNS disease. Steroid or plasmapheresis are used in treatment of cerebral vasculitis. We experienced a case of 9-year-old boy who had presented with Henoch-Schonlein purpura nephritis complicating encephalopathy accompanied by hypertension and cerebral vasculitis. Brain MRI showed multiple small nodular-linear pattern enhancing lesions in whole cerebral hemispheres and focal increased T2 signal in the right basal ganglia. We used intravenous immunoglobulin in treatment of cerebral vasculitis. We report this case with a brief review of related literature.

• 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.