急性手指缺血：抗合成酶综合征的一种罕见表现。
Acute digital ischemia: A rare presentation of antisynthetase syndrome.
作者信息
Chan Jin Ei, Palakodeti Sandeep, Koster Matthew J
机构信息
Department of Medicine, Fudan University Shanghai Medical College, Shanghai, China.
Division of Hospital Internal Medicine, Department of Internal Medicine, Mayo Clinic College of Medicine and Science, Minnesota, USA.
出版信息
Eur J Rheumatol. 2017 Mar;4(1):63-65. doi: 10.5152/eurjrheum.2017.16077. Epub 2017 Mar 1.
Antisynthetase syndrome (ASS) is recognized as a subgroup of idiopathic inflammatory myopathies (IIMs). It is associated with autoantibodies directed against aminoacyl-transfer ribonucleic acid (tRNA) synthetase enzymes. We report the first case of anti-PL-7/anti-SSA 52kD ASS presenting as acute digital ischemia, an association not described previously. Occlusive vasculopathy is a rare but serious manifestation that can be seen at presentation in patients with ASS and may herald the onset of severe interstitial lung disease (ILD). Comprehensive evaluation should be performed to confirm the presence of subclinical myositis. Extensive myositis-specific antibody testing is strongly recommended even if initial screening autoimmune serologies are unrevealing.
抗合成酶综合征（ASS）被认为是特发性炎性肌病（IIM）的一个亚组。它与针对氨酰基转移核糖核酸（tRNA）合成酶的自身抗体有关。我们报告了首例以急性指端缺血为表现的抗PL - 7/抗SSA 52kD抗合成酶综合征，此前未见这种关联的描述。闭塞性血管病是一种罕见但严重的表现，可见于抗合成酶综合征患者的初发症状中，可能预示着严重间质性肺病（ILD）的发作。应进行全面评估以确认亚临床肌炎的存在。即使初始筛查自身免疫血清学检查未发现异常，也强烈建议进行广泛的肌炎特异性抗体检测。
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