Cough and Nasal Pain: Putting Together an Uncommon Diagnosis

E. Jensen, P. Panse, K. Cummings, C. Jokerst, M. Gotway
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Abstract

The pulmonary vasculitides include a wide spectrum of disorders that have in common inflammation and destruction of pulmonary blood vessels. Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is the most common of the antineutrophil cytoplasmic antibody–associated small-vessel vasculitides, and affects a number of organ systems. GPA affects a wide age range, although it typically presents in middle adulthood. The classic triad of GPA—upper airway involvement (sinusitis, otitis media, mucosal ulcerations, airway inflammation), lower respiratory tract disease, and glomerulonephritis—is not always evident at presentation. Furthermore, limited forms of GPA occur, in which the expected upper airway and renal involvement may be lacking. The imaging manifestations of GPA commonly include multiple, bilateral nodules or masses, frequently with cavitation or necrosis. Diffuse lung opacity due to alveolar hemorrhage, or tracheobronchial inflammation that may result in stenosis, may be encountered. Systemic GPA may be diagnosed when cytoplasmic antineutrophil cytoplasmic antibody/anti-proteinase 3 antibodies are detected in the proper clinical context, but, often, the diagnosis of GPA relies on recognition of a combination of common clinical, laboratory, and imaging findings with typical histopathologic changes noted from a biopsy of the affected tissue. A combination of a corticosteroid and immunomodulatory agents is used to induce and maintain remission in patients with GPA. Although potentially fatal if untreated, immunosuppressive therapy has dramatically improved survivorship in patients with GPA.
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咳嗽和鼻痛:把一个不常见的诊断放在一起
肺血管炎包括广泛的疾病,常见的炎症和肺血管破坏。多血管炎肉芽肿病(GPA),以前称为韦格纳肉芽肿病,是最常见的抗中性粒细胞细胞质抗体相关的小血管增生,并影响许多器官系统。GPA影响的年龄范围很广,尽管它通常出现在中年。典型的gpa三联征——上呼吸道受累(鼻窦炎、中耳炎、粘膜溃疡、气道炎症)、下呼吸道疾病和肾小球肾炎——在表现时并不总是很明显。此外,有限形式的GPA发生,其中可能缺乏预期的上呼吸道和肾脏受累。GPA的影像学表现通常为多发双侧结节或肿块,常伴有空化或坏死。可能会遇到肺泡出血引起的弥漫性肺混浊,或可能导致狭窄的气管支气管炎症。当在适当的临床背景下检测到细胞质抗中性粒细胞抗体/抗蛋白酶3抗体时,可以诊断出系统性GPA,但通常,GPA的诊断依赖于对常见临床、实验室和影像学结果的综合认识,以及受影响组织的活检所记录的典型组织病理学变化。皮质类固醇和免疫调节剂的组合用于诱导和维持GPA患者的缓解。虽然免疫抑制治疗如果不治疗可能致命,但可以显著提高GPA患者的生存率。
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Clinical Pulmonary Medicine
Clinical Pulmonary Medicine Medicine-Critical Care and Intensive Care Medicine
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期刊介绍: Clinical Pulmonary Medicine provides a forum for the discussion of important new knowledge in the field of pulmonary medicine that is of interest and relevance to the practitioner. This goal is achieved through mini-reviews on focused sub-specialty topics in areas covered within the journal. These areas include: Obstructive Airways Disease; Respiratory Infections; Interstitial, Inflammatory, and Occupational Diseases; Clinical Practice Management; Critical Care/Respiratory Care; Colleagues in Respiratory Medicine; and Topics in Respiratory Medicine.
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