• Journal of Medicine and Life Science
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• 제21권1호
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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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• 제33권1호
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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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• 제43권5호
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• pp.786-791
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• 1996

저자들은 흉통을 주소로 내원하여 흉막 조직검사상 leukocytoclastic vasculitis로 확진된 혈관염에 의한 양측성 흉막염이 발생한 공피증 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• 대한영상의학회지
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• 제82권4호
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• pp.791-807
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• 2021

혈관염은 혈관벽의 염증을 특징으로 하는 드문 전신 질환으로 간혹 미만성 폐출혈이나 급성사구체신염으로 생명을 위협하기도 한다. 원인을 알 수 없는 일차성 혈관염도 있지만 자가면역 질환이나 약물, 감염, 종양 등 수많은 원인들에 의해 발생하는 이차성 혈관염도 있으며 분류가 복잡하고 명확한 진단검사가 없어 진단에 어려움이 있다. 또한 다양하고 비특이적인 증상과 징후 및 검사 소견을 보여 혈관염의 진단에는 임상양상, 영상 검사, 자가항체 검사, 병리소견 등 여러 가지 결과를 종합하는 것이 필수적이다. 이 종설에서는 혈관염의 분류와 진단에 중요한 증상 및 징후, 특징적으로 폐를 침범하는 대표적인 폐혈관염의 영상 소견과 감별진단, 그리고 새로운 혈관염 분류를 위한 국제적 연구인 Diagnostic and Classification Criteria in Vasculitis에 대해 소개하고자 한다.

• Tuberculosis and Respiratory Diseases
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• 제74권4호
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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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• 제25권1호
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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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• 제63권5호
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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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• 제79권4호
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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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• 제46권10호
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• pp.1040-1043
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• 2003

저자들은 9세 남아에서 고혈압과 뇌혈관염이 동반된 상태에서 뇌증을 보인 HSP 신염 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제45권12호
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• pp.1601-1605
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• 2002

본 저자들은 경련과 의식저하를 보인 알레르기성 자반증 환아에서 신경학적 합병증으로 대뇌 동맥의 혈관염이 발생된 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.