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慢性无症状性弥漫性肺泡出血合并显微镜下多血管炎:一例报告并文献复习

Chronic and Asymptomatic Diffuse Alveolar Haemorrhage with Microscopic Polyangiitis: A Case Report and Review of the Literature.

作者信息

Tashiro Hiroki, Takahashi Koichiro, Sadamatsu Hironori, Uchida Masaru, Kimura Shinya, Sueoka-Aragane Naoko

机构信息

Division of Haematology, Respiratory Medicine and Oncology, Department of Internal Medicine, Faculty of Medicine, Saga University, 5-1-1 Nabeshima, Saga 849-8501, Japan; Department of Internal Medicine, Karatsu Red Cross Hospital, 2430 Watada, Karatsu-shi, Saga 847-8588, Japan.

Division of Haematology, Respiratory Medicine and Oncology, Department of Internal Medicine, Faculty of Medicine, Saga University, 5-1-1 Nabeshima, Saga 849-8501, Japan.

出版信息

Case Rep Rheumatol. 2016;2016:1658126. doi: 10.1155/2016/1658126. Epub 2016 Dec 5.

DOI:10.1155/2016/1658126
PMID:28050304
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5165133/
Abstract

Diffuse alveolar haemorrhage (DAH) is one of the major causes of death in microscopic polyangiitis (MPA) patients, because of acute respiratory failure with various respiratory symptoms. We, herein, present a case of chronic and asymptomatic DAH in a patient with MPA who was diagnosed by fibreoptic bronchoscopy. The patient showed localized reticular shadows, without any respiratory symptoms, and absence of inflammatory reactions, such as fever and CRP elevation, which is atypical for DAH. Three months after appearance of the lung abnormalities, DAH with MPA was diagnosed by fibreoptic bronchoscopy. She was initially treated with only corticosteroids and has thereafter been maintained with corticosteroids and azathioprine without relapse to date. We reviewed the literature for similar cases and opined that physicians should perform fibreoptic bronchoscopy in MPA patients with chronic lung abnormalities and anaemia to identify DAH, even if the patients show no respiratory symptoms and in the absence of inflammatory reactions.

摘要

弥漫性肺泡出血(DAH)是显微镜下多血管炎(MPA)患者的主要死亡原因之一,因其会导致伴有各种呼吸道症状的急性呼吸衰竭。在此,我们报告一例MPA患者出现慢性无症状性DAH的病例,该病例通过纤维支气管镜检查得以确诊。患者表现为局限性网状阴影,无任何呼吸道症状,且无发热和CRP升高等炎症反应,这在DAH中并不典型。肺部异常出现三个月后,通过纤维支气管镜检查诊断为MPA合并DAH。她最初仅接受糖皮质激素治疗,此后一直使用糖皮质激素和硫唑嘌呤维持治疗,至今未复发。我们查阅了相关文献以寻找类似病例,并认为医生应对有慢性肺部异常和贫血的MPA患者进行纤维支气管镜检查,以确定是否存在DAH,即便患者没有呼吸道症状且无炎症反应。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/182e/5165133/5ec69ce40b55/CRIRH2016-1658126.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/182e/5165133/29969f8ab464/CRIRH2016-1658126.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/182e/5165133/df5edfa55cb6/CRIRH2016-1658126.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/182e/5165133/5ec69ce40b55/CRIRH2016-1658126.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/182e/5165133/29969f8ab464/CRIRH2016-1658126.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/182e/5165133/df5edfa55cb6/CRIRH2016-1658126.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/182e/5165133/5ec69ce40b55/CRIRH2016-1658126.003.jpg

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Arthritis Rheumatol. 2016 Jun;68(6):1467-76. doi: 10.1002/art.39562.
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Evaluation and validation of biomarkers in granulomatosis with polyangiitis and microscopic polyangiitis.肉芽肿性多血管炎和显微镜下多血管炎生物标志物的评估与验证
Nephrol Dial Transplant. 2016 Jun;31(6):930-6. doi: 10.1093/ndt/gfv336. Epub 2015 Sep 26.
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Diffuse pulmonary hemorrhage: clues to the diagnosis.
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Intern Med. 2013;52(1):5-13. doi: 10.2169/internalmedicine.52.8863. Epub 2013 Jan 1.
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