Rheumatology Department, Westmead Hospital, Sydney, New South Wales, Australia. Email:
Intern Med J. 2011 Feb;41(2):197-9. doi: 10.1111/j.1445-5994.2010.02381.x.
In a patient with early topoisomerase antibody-positive scleroderma, antinuclear antibody positivity was fortuitously observed to predate nailfold capillaroscopy changes. Using this case as a template, the prediagnostic phase of the presumed multifactorial disease may be divided into 5 temporal phases--phase 1 representing conception and intrauterine environment, phase 2 representing the extrauterine environment predating environmental exposure; phase 3 representing the early post-environmental exposure interval with no detectable perturbed body status; phase 4 representing the post-environmental exposure interval characterized by autoantibody production and microvascular changes, and phase 5, the symptomatic clinical prediagnostic interval (Raynaud's, skin, musculoskeletal, gastrointestinal, cardiorespiratory) prompting scleroderma diagnosis. Temporal classification of prescleroderma aids in both the understanding and definition of scleroderma 'onset'. If altered nailfold capillaries and autoantibodies develop at comparable rates, and if the findings from this case--that autoantibody changes precede microvascular changes--are truly representative of the preclinical disease phase, then these findings argue that the evolution of the disease is from within the vessel outwards, rather than vice versa.
在一位早期拓扑异构酶抗体阳性硬皮病患者中，偶然观察到抗核抗体阳性先于甲襞毛细血管镜改变。以此病例为模板，假定的多因素疾病的预测诊断阶段可分为 5 个时间阶段——第 1 阶段代表受孕和宫内环境，第 2 阶段代表环境暴露前的子宫外环境；第 3 阶段代表早期环境暴露后无明显躯体状态紊乱的间隔期；第 4 阶段代表以自身抗体产生和微血管改变为特征的环境暴露后间隔期，第 5 阶段为有症状的临床预测诊断间隔期（雷诺现象、皮肤、肌肉骨骼、胃肠道、心肺）提示硬皮病诊断。硬皮病前的时间分类有助于理解和定义硬皮病“发病”。如果改变的甲襞毛细血管和自身抗体以可比的速度发展，如果这个病例的发现——自身抗体变化先于微血管变化——真的代表了临床前疾病阶段，那么这些发现表明疾病的演变是从血管内部向外，而不是相反。
