small-vessel vasculitis

  • 文章类型: Case Reports
    白细胞碎裂性血管炎(LCV)是一种小血管血管炎,其特征是炎症和对小血管壁的损害。它通常表现为可触及的紫癜,并可能与各种全身状况有关。虽然其病因多样,LCV与全身性疾病相关,感染,药物,和自身免疫性疾病。这里,我们介绍了1例失代偿性心力衰竭患者的LCV病例.一名58岁男子的下肢和阴囊肿胀逐渐恶化,持续的干咳与胫骨上的轻微溃疡性病变有关,和斑片状皮疹,有脓疱和扁平的红色斑点。他三天前因心房颤动和快速心室率住院,他开始服用胺碘酮。这种皮疹持续了三天,然而他否认有任何不适或瘙痒。根据他的临床表现,实验室评估,和影像学发现,他被诊断为胺碘酮诱导的LCV。
    Leukocytoclastic vasculitis (LCV) is a small-vessel vasculitis characterized by inflammation and damage to the walls of small blood vessels. It typically presents with palpable purpura and can be associated with various systemic conditions. Although its etiology is diverse, LCV has been associated with systemic diseases, infections, medications, and autoimmune disorders. Here, we present a case of LCV in a patient with decompensated heart failure. A 58-year-old man presented with progressively deteriorating swelling in both his lower limbs and scrotum, a persistent dry cough associated with minor ulcerative lesions on his shins, and a patchy rash with pustules and flat reddish spots. He was hospitalized three days prior due to atrial fibrillation and rapid ventricular rate, for which he was commenced on amiodarone. This rash persisted for three days, yet he denied experiencing any discomfort or itchiness along with it. Based on his clinical picture, laboratory evaluations, and imaging findings, he was diagnosed with LCV induced by amiodarone.
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  • 文章类型: Case Reports
    白细胞碎裂性血管炎(超敏反应性血管炎)被定义为由于感染引起的皮肤或其他全身表现的小血管炎症,毒品,或肿瘤性疾病。该临床病例报告强调了头孢曲松与白细胞碎裂性血管炎之间的关联,该患者49岁,有青霉素过敏史。米氮平治疗焦虑症.还综述了有关抗生素诱导的白细胞碎裂性血管炎的文章。患者在5天前报告了上呼吸道感染和发烧的症状,为此她接受了头孢曲松2天,然后到急诊科就诊,上肢和下肢出现瘙痒性皮疹,嘴唇肿胀1天。皮疹是红斑,斑丘疹,发痒,和非招标,没有粘膜受累.实验室检查显示白细胞增多(白细胞,22.3×109/L),主要为嗜酸性粒细胞(18.4%)。经验停止头孢曲松后,患者给予泼尼松龙和抗组胺药。皮肤活检证实了白细胞碎裂性血管炎的诊断。治疗开始后观察到显著的临床改善。在后续行动中,皮疹完全缓解,没有疤痕;然而,下肢有脱皮现象。识别抗生素诱导的白细胞碎裂性血管炎至关重要,因为许多类别的抗生素都可能导致这种情况。继续服用有问题的药物可能会导致危及生命的并发症。
    Leukocytoclastic vasculitis (hypersensitivity vasculitis) is defined as small blood vessel inflammation with skin or other systemic manifestations due to infections, drugs, or neoplastic disease. This clinical case report highlights an association between ceftriaxone and leukocytoclastic vasculitis in a 49-year-old female patient with a history of penicillin allergy, on mirtazapine for anxiety disorder. Articles concerning antibiotic-induced leukocytoclastic vasculitis are also reviewed. The patient reported a symptom of upper respiratory tract infection and fever 5 days previously for which she received ceftriaxone for 2 days before presenting to the emergency department with a pruritic skin rash in the upper and lower extremities and swollen lips for 1 day. The rash was erythematous, maculopapular, itchy, and non-tender, with no mucus membrane involvement. Laboratory investigations revealed leukocytosis (white blood cells, 22.3 × 109/L) that was mainly eosinophilic (18.4%). The patient was administered prednisolone and antihistamine after stopping ceftriaxone empirically. A skin biopsy confirmed the diagnosis of leukocytoclastic vasculitis. Significant clinical improvement was observed after treatment initiation. Upon follow-up, the skin rash was resolved entirely with no scars; however, there was skin-peeling over the lower extremities. Recognition of antibiotic-induced leukocytoclastic vasculitis is crucial as many classes of antibiotics can contribute to this condition. Continuation of the offending drug may lead to life-threatening complications.
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  • 文章类型: Journal Article
    Background: IgA vasculitis/Henoch-Schoenlein purpura (IgAV/HSP) is a systemic small vessel vasculitis of unknown pathogenesis predominantly affecting children. While skin, GI tract, joints, and kidneys are frequently affected and considered, central nervous system (CNS) involvement of this disease is underestimated. Methods: We provide a case report and systematically review the literature on IgAV, collecting data on the spectrum of neurological manifestations. Results: We report on a 7-year-old girl with IgAV who presented with diplopia and afebrile focal seizures, which preceded the onset of purpura. Cranial magnetic resonance imaging was consistent with posterior reversible encephalopathy syndrome (PRES), showing typical focal bilateral parietal swelling and cortical and subcortical high signal intensities on T2-fluid attenuated inversion recovery (FLAIR) images predominantly without diffusion restriction. Cerebrospinal fluid analysis and blood tests excluded systemic inflammation or vasculitis. Interestingly, hypertension was not a hallmark of the developing disease in the initial phase of PRES manifestation. Renal disease and other secondary causes for PRES were also excluded. Supportive- and steroid treatment resulted in restitution ad integrum. Reviewing the literature, we identified 28 other cases of IgAV with CNS involvement. Severe CNS involvement includes seizures, cerebral edema, or hemorrhage, as well as PRES. Thirteen patients fulfilled all diagnostic criteria of PRES. The mean age was 11.2 years (median 8.0, range 5-42 years), with no reported bias toward gender or ethnic background. Treatment regimens varied from watchful waiting to oral and intravenously steroids up to plasmapheresis. Three cases showed permanent CNS impairment. Conclusion: Collectively, our data demonstrate that (I) severe CNS involvement such as PRES is an underappreciated feature of IgAV, (II) CNS symptoms may precede other features of IgAV, (III) PRES can occur in IgAV, and differentiation from CNS vasculitis is challenging, (IV) pathogenesis of PRES in the context of IgAV remains elusive, which hampers treatment decisions. We, therefore, conclude that clinical awareness and the collection of structured data are necessary to elucidate the pathophysiological connection of IgAV and PRES.
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  • 文章类型: Journal Article
    背景:中主动脉综合征是一种罕见疾病。有几种手术治疗方法可用;然而,最佳治疗策略和长期结局仍未阐明.我们在此报告了6例接受解剖外搭桥手术治疗的中主动脉综合征患者的5年结局。
    方法:在2013年至2016年之间,我们研究所有6例患者接受了中主动脉综合征的解剖外搭桥:3例患有Takayasu的动脉炎,其中一人患有血管血管炎,两名患有不明原因的中主动脉增生综合征。患者包括五名女性和一名男性,平均年龄59.7岁.四名患者的高血压不受控制,正在接受抗高血压药物治疗。平均踝臂压指数为.61。3例大动脉炎患者因充血性心力衰竭住院。这些患者接受了从降主动脉到肾下腹主动脉的搭桥手术,其中一人还接受了心脏手术。显微镜下多血管炎患者接受了Y型移植和主动脉瘤切除术。随后,旁路手术从降主动脉经膈至移植物.两名原因不明的患者接受了从近端降主动脉到远端降主动脉的旁路手术。在5年的随访中没有早期或晚期死亡。在随访期间,我们没有观察到吻合部位狭窄的任何变化或新的动脉瘤变化。在所有情况下,抗高血压药物的数量都减少了,和严重的症状,包括头痛,严重的腹痛,跛行,心力衰竭,改善所有患者。踝臂压指数增加到1.11,并且五年没有变化。肾功能保持稳定,随访时脑钠肽水平从302.8降至74.5pg/mL。
    结论:解剖外分流术治疗中主动脉综合征是安全有效的,可以帮助预防肾衰竭,缓解严重的缺血症状.
    BACKGROUND: Middle aortic syndrome is a rare disease. Several surgical treatments are available; however, the optimal treatment strategy and long-term outcomes remain unelucidated. We herein report the 5-year outcomes of six patients treated with extra-anatomical bypass surgery for middle aortic syndrome.
    METHODS: Between 2013 and 2016, six patients underwent extra-anatomical bypass for middle aortic syndrome at our institute: three had Takayasu\'s arteritis, one had vessel vasculitis, and two had middle aortic hypoplastic syndrome of unknown origin. The patients included five women and one man, with a mean age of 59.7 years. Four patients had uncontrolled hypertension and were receiving antihypertensive medications. The mean ankle-brachial pressure index was .61. The three patients with Takayasu\'s arteritis were hospitalized for congestive heart failure. These patients underwent bypass surgery from the descending aorta to the infrarenal abdominal aorta, and one also underwent concomitant heart surgery. The patient with microscopic polyangiitis underwent Y-grafting with an aortic aneurysmectomy. Subsequently, bypass surgery was performed from the descending aorta to the graft via the diaphragm. The two patients with unknown causes underwent bypass surgery from the proximal descending aorta to the distal descending thoracic aorta. There were no early or late deaths at the 5-year follow-up. We did not observe any changes in anastomotic site stenosis or new aneurysmal changes during the follow-up period. The number of antihypertensive medications was reduced in all cases, and critical symptoms, including headache, severe abdominal pain, claudication, and heart failure, improved in all patients. The ankle-brachial pressure index increased to 1.11 and did not change for five years. Renal function remained stable, and the brain natriuretic peptide level decreased from 302.8 to 74.5 pg/mL at follow-up.
    CONCLUSIONS: Extra-anatomical bypass for middle aortic syndrome is safe and effective, and can help prevent renal failure, and relieve critical ischemic symptoms.
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  • 文章类型: Case Reports
    Drug-induced skin reactions are common, but only a small portion (10%) are attributed to a vasculitic mechanism. Small-vessel vasculitis (SVV) with leukocytoclastic histopathology is usually described in drug-induced vasculitis; however, given the shared histopathologic features between drug-induced vasculitis and other SVV, it is crucial to rule out infectious or other autoimmune etiologies underlying the clinical presentation. We hereby sought to present a case of sulfonamide-induced leukocytoclastic vasculitis, limited to the skin, in a patient with Ehlers-Danlos syndrome in order to emphasize the need for a broad diagnostic and clinical exclusion workup.
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