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本文引用的文献

1
Autoantibodies against bactericidal/permeability-increasing protein in patients with cystic fibrosis.囊性纤维化患者中针对杀菌/通透性增加蛋白的自身抗体。
QJM. 1996 Apr;89(4):259-65. doi: 10.1093/qjmed/89.4.259.
2
Bactericidal/permeability-increasing protein protects vascular endothelial cells from lipopolysaccharide-induced activation and injury.杀菌/通透性增加蛋白可保护血管内皮细胞免受脂多糖诱导的激活和损伤。
Infect Immun. 1994 Sep;62(9):3930-6. doi: 10.1128/iai.62.9.3930-3936.1994.
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Arthritis in cystic fibrosis.囊性纤维化中的关节炎
Arch Dis Child. 1984 Apr;59(4):377-9. doi: 10.1136/adc.59.4.377.
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Pathogenesis and management of arthropathy in cystic fibrosis.囊性纤维化关节病的发病机制与管理
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Episodic arthropathy in adult cystic fibrosis.成人囊性纤维化中的发作性关节病
Q J Med. 1987 Aug;64(244):651-9.
6
Purpuric rashes in cystic fibrosis.
Am J Dis Child. 1989 Sep;143(9):1030-2. doi: 10.1001/archpedi.1989.02150210056018.
7
Vasculitis complicating cystic fibrosis.血管炎并发囊性纤维化。
Q J Med. 1989 Jul;72(267):609-21.
8
Vasculitis and arthropathy in cystic fibrosis.囊性纤维化中的血管炎和关节病。
J R Soc Med. 1992;85 Suppl 19(Suppl 19):38-40.
9
Hypergammaglobulinemic purpura in cystic fibrosis.囊性纤维化中的高球蛋白血症性紫癜
Acta Paediatr Scand. 1978 Jul;67(4):443-7. doi: 10.1111/j.1651-2227.1978.tb16351.x.

囊性纤维化中的复发性血管炎。

Recurrent vasculitis in cystic fibrosis.

作者信息

Wujanto Lareina, Ross Clare

机构信息

Dermatology Department, Ealing Hospital, Southall, Middlesex, UK.

出版信息

BMJ Case Rep. 2010 Oct 22;2010:bcr0320102834. doi: 10.1136/bcr.03.2010.2834.

DOI:10.1136/bcr.03.2010.2834
PMID:22791575
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3027361/
Abstract

A 22-year-old man presented with recurrent palpable purpuric rash. His clubbing relates to underlying cystic fibrosis (CF) and his rash was identified as CF-related vasculitis, a rare extrapulmonary manifestation of the disease. It occurs predominantly on the lower limbs, mainly over the dorsa of the feet, ankles and tibial surfaces. The rash occurred while the patient had an infective exacerbation of CF (IECF), however, there had also been previous occurrences without worsening of his pulmonary symptoms, to which the rash remitted spontaneously. The patient responded well to immunosuppression, which was given on this admission due to worsening of his CF-related vasculitis. He died 18 months within the onset of his initial rash.

摘要

一名22岁男性出现反复可触及的紫癜性皮疹。他的杵状指与潜在的囊性纤维化(CF)有关,其皮疹被诊断为CF相关血管炎,这是该疾病一种罕见的肺外表现。皮疹主要出现在下肢,主要分布在足背、脚踝和胫前表面。皮疹在患者CF感染加重期(IECF)出现,但之前也有过发作,且肺部症状并未加重,皮疹可自行消退。由于CF相关血管炎恶化,此次入院给予免疫抑制治疗,患者反应良好。他在初次出现皮疹后的18个月内死亡。