孤立性保奇免疫性肺毛细血管炎的致命结局:病例报告

IF 0.8 Q4 RESPIRATORY SYSTEM
Respirology Case Reports Pub Date : 2024-10-17 eCollection Date: 2024-10-01 DOI:10.1002/rcr2.70051
Zeinab El Mawla, Ghinwa Hammoud, Racha Abed El Hamid, Abbas Zreik, Ali Tfayli, Bassam Mansour
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引用次数: 0

摘要

孤立性保济免疫性肺毛细血管炎(IPIPC)是一种罕见的小血管炎,只影响肺部,引起肺毛细血管炎症,可能导致肺泡出血等严重后果。一名 23 岁的女性患者曾被诊断为类风湿性关节炎,出现咯血和呼吸困难,最终被诊断为 IPIPC。尽管她接受了大剂量类固醇和静脉注射免疫球蛋白治疗,但病情仍不断恶化,最终导致呼吸衰竭而死亡。IPIPC 通常没有全身症状,ANCA 阳性,使诊断和治疗复杂化。影像学检查、支气管镜检查和组织病理学检查是诊断的关键,而治疗方法通常包括皮质类固醇激素和可能的免疫抑制剂。该病例强调了鉴别和治疗 IPIPC 所面临的挑战,突出了早期干预对改善预后的重要性,尽管并发症仍可能导致严重的呼吸系统问题和死亡。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Fatal outcome in isolated Pauci-immune pulmonary capillaritis: A case report.

Isolated Pauci-immune pulmonary capillaritis (IPIPC) is a rare form of small vessel vasculitis that affects only the lungs, causing inflammation of pulmonary capillaries and potentially leading to severe outcomes like alveolar haemorrhage. A 23-year-old woman with a prior diagnosis of rheumatoid arthritis presented with hemoptysis and respiratory distress, ultimately diagnosed with IPIPC. Despite treatment with high-dose steroids and intravenous immunoglobulin, her condition deteriorated, resulting in respiratory failure and death. IPIPC often lacks systemic symptoms and ANCA positivity, complicating diagnosis and treatment. Imaging, bronchoscopy, and histopathology are key for diagnosis, while management typically involves corticosteroids and possibly immunosuppressives. The case underscores the challenges in identifying and treating IPIPC, highlighting the importance of early intervention to improve prognosis, even though complications can still lead to significant respiratory issues and mortality.

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来源期刊
Respirology Case Reports
Respirology Case Reports RESPIRATORY SYSTEM-
CiteScore
1.40
自引率
0.00%
发文量
178
审稿时长
8 weeks
期刊介绍: Respirology Case Reports is an open-access online journal dedicated to the publication of original clinical case reports, case series, clinical images and clinical videos in all fields of respiratory medicine. The Journal encourages the international exchange between clinicians and researchers of experiences in diagnosing and treating uncommon diseases or diseases with unusual presentations. All manuscripts are peer-reviewed through a streamlined process that aims at providing a rapid turnaround time from submission to publication.
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