Identification of clinical features and autoantibodies associated with calcinosis in dermatomyositis.
Identification of clinical features and autoantibodies associated with calcinosis in dermatomyositis.
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DOI：
10.1001/jamadermatol.2013.10416
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发表时间：
2014-07
期刊：
影响因子：
10.9
通讯作者：
Fiorentino, David
中科院分区：
文献类型：
--
作者：
Valenzuela, Antonia;Chung, Lorinda;Casciola-Rosen, Livia;Fiorentino, David
先前的研究估计，高达20%的成年皮肌炎（DM）有钙质沉着症，这可能导致显着的发病率。确定危险因素可能会提供一个更好的理解的发病机制，并最终治疗这一困难的临床问题。糖尿病成人钙质沉着症的危险因素尚未得到广泛研究。确定钙沉着症的患病率，并确定广泛表型成人DM队列的相关临床特征。对2006年1月1日至2013年1月1日诊断为DM的126例患者进行了横断面研究。患者为在斯坦福大学医学中心门诊就诊的成人（≥18岁）。钙质沉着症，定义为体格检查时皮肤和皮下组织中存在钙沉积。14例患者（11.1%）有钙质沉着，最常见的是四肢。与无钙质沉着的患者相比，有钙质沉着的患者病程更长（中位数，6.9年;范围，2.4-18.1年; vs中位数，3.9年;范围，0.2-19.2年; P = 0.003），指尖溃疡更多（50.0% vs 9.3%，P <0.001）。钙质沉着症与间质性肺病和抗MDA-5自身抗体之间的相关性被确定，但这种相关性在校正指尖溃疡的多变量模型中并不存在。在所有多变量模型中，指尖溃疡和疾病持续时间与钙质沉着症密切相关，与潜在的自身抗体无关。在针对指尖溃疡和其他协变量进行调整的多变量分析中，针对NXP-2的自身抗体与钙质沉着症相关（比值比，15.52; 95%CI，2.01-119.90），而抗转录中间因子1-γ抗体具有保护作用（比值比，0.2; 95%CI，0.01-0.99）。钙质沉着症是一个相对罕见的临床特征，在我们的队列成人糖尿病。我们的数据表明，钙质沉着症与病程延长、指尖溃疡和NXP-2自身抗体呈正相关，与转录中介因子1-γ抗体呈负相关。糖尿病患者钙质沉着和指尖溃疡的发生可能与一种共同的血管机制有关。
Prior studies have estimated that up to 20% of adults with dermatomyositis (DM) have calcinosis, which can lead to significant morbidity. Identification of risk factors may provide a better understanding of the pathogenesis and ultimately therapy for this difficult clinical problem. Risk factors for calcinosis in adults with DM have not been extensively studied. To determine the prevalence of calcinosis and to identify associated clinical features in a cohort of extensively phenotyped adults with DM. A cross-sectional study of 126 patients diagnosed as having DM from January 1, 2006, through January 1, 2013, was performed. Patients were adults (≥18 years of age) attending the Stanford University Medical Center clinic. Calcinosis, defined as the presence of calcium deposition in the skin and subcutaneous tissues on physical examination. Fourteen patients (11.1%) had calcinosis, with the extremities most commonly involved. Patients with vs those without calcinosis had a longer disease duration (median, 6.9 years; range, 2.4–18.1; vs median, 3.9 years; range, 0.2-19.2 years; P = .003) and more fingertip ulcers (50.0% vs 9.3%, P < .001). An association between calcinosis and both interstitial lung disease and anti–MDA-5 autoantibodies was identified, but this association did not persist in multivariate models that adjusted for fingertip ulcers. Fingertip ulcers and disease duration were strongly associated with calcinosis in all multivariate models, independent of the underlying autoantibody present. Autoantibodies to NXP-2 were associated with calcinosis (odds ratio, 15.52; 95% CI, 2.01-119.90), whereas anti–transcriptional intermediary factor 1-γ antibodies were protective (odds ratio, 0.2; 95% CI, 0.01-0.99) in multivariate analyses that adjusted for fingertip ulcers and other covariates. Calcinosis was a relatively uncommon clinical feature in our cohort of adults with DM. Our data suggest that calcinosis is positively associated with longer disease duration, fingertip ulcers, and NXP-2 autoantibodies and negatively associated with transcriptional intermediary factor 1-γ antibodies. A common vascular mechanism may underlie the development of both calcinosis and fingertip ulcers in patients with DM.
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作者：
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