Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, California.
Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, California2Department of Dermatology, Stanford University School of Medicine, Stanford, California.
JAMA Dermatol. 2014 Jul;150(7):724-9. doi: 10.1001/jamadermatol.2013.10416.
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.
DESIGN, SETTING, AND PARTICIPANTS: 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.
先前的研究估计，多达 20%的皮肌炎（DM）成人患者存在钙沉积，这可能导致严重的发病率。确定风险因素可以更好地了解这一临床难题的发病机制，并最终为其提供治疗方法。DM 成人患者钙沉积的风险因素尚未得到广泛研究。
在一组广泛表型的 DM 成年患者中，确定钙沉积的患病率，并确定相关的临床特征。
设计、设置和参与者：对 2006 年 1 月 1 日至 2013 年 1 月 1 日期间被诊断为患有 DM 的 126 例患者进行了一项横断面研究。患者为成年（≥18 岁），在斯坦福大学医疗中心诊所就诊。
钙沉积定义为体格检查时皮肤和皮下组织中钙沉积的存在。
14 例（11.1%）患者存在钙沉积，四肢最常受累。与无钙沉积的患者相比，有钙沉积的患者疾病病程更长（中位数为 6.9 年；范围为 2.4-18.1；vs 中位数为 3.9 年；范围为 0.2-19.2 年；P = .003），指尖溃疡更多（50.0% vs 9.3%，P < .001）。钙沉积与间质性肺病和抗 MDA-5 自身抗体之间存在关联，但在调整指尖溃疡的多变量模型中，这种关联并未持续存在。在所有多变量模型中，指尖溃疡和疾病病程与钙沉积密切相关，而与存在的基础自身抗体无关。NXP-2 自身抗体与钙沉积相关（比值比，15.52；95%置信区间，2.01-119.90），而转录中介因子 1-γ 抗体在调整指尖溃疡和其他协变量的多变量分析中具有保护作用（比值比，0.2；95%置信区间，0.01-0.99）。
钙沉积是我们 DM 成年患者队列中相对罕见的临床特征。我们的数据表明，钙沉积与疾病病程较长、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。血管的共同机制可能是导致 DM 患者钙沉积和指尖溃疡的共同原因。
