Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy.
Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.
Autoimmun Rev. 2017 Mar;16(3):253-257. doi: 10.1016/j.autrev.2017.01.008. Epub 2017 Jan 29.
Arthritis, myositis and interstitial lung disease (ILD) constitute the classic clinical triad of anti-synthetase syndrome (ASSD). These patients experience other accompanying features, such as Raynaud's phenomenon, fever or mechanic's hands. Most ASSD patients develop the complete triad during the follow-up. In the present study we aimed to determine whether the subsequent appearance of accompanying features may suggest the development of triad findings lacking at the onset in anti-Jo1 positive ASSD patients.
Anti-Jo1 positive patients presenting with incomplete ASSD (no >2 classic triad features) were assessed. Clinical characteristics and clusters of disease manifestations were retrospectively collected and analyzed in a large international multicenter cohort of ASSD patients.
165 patients (123 women) with incomplete ASSD were identified. Ninety-five patients (57.5%) developed new classic triad manifestations after 15months median (IQR 9-51) and 40 (24%) developed new accompanying features after 19months median (IQR 6-56) from disease onset. During the follow-up, the ex-novo occurrence of triad features was observed in 32 out of 40 patients (80%) with new accompanying findings and in 63 out of 125 patients (50.5%) without new accompanying findings (p=0.002). In patients with at least one new accompanying feature the odds ratio for the occurrence of new triad manifestations was 3.94 with respect to patients not developing ex-novo accompanying findings (95% CI 1.68-9.21, p=0.002).
Anti-Jo1 ASSD patients with incomplete forms at disease onset are at high risk for the subsequent occurrence of lacking classic triad findings. Although all ASSD patients should be carefully assessed for the occurrence of new triad features, a closer follow-up should be considered in the subgroup of patients developing ex novo accompanying findings. These patients, indeed, have near four-fold increased risk for new classic triad manifestation occurrence with respect to patients not presenting ex novo accompanying findings.
关节炎、肌炎和间质性肺病（ILD）构成抗合成酶综合征（ASSD）的经典临床三联征。这些患者还伴有其他伴随特征，如雷诺现象、发热或技工手。大多数 ASSD 患者在随访过程中会出现完整的三联征。本研究旨在确定后续出现的伴随特征是否可能提示抗-Jo1 阳性 ASSD 患者在发病时缺乏三联征表现。
评估了表现为不完全 ASSD（无>2 个经典三联征特征）的抗-Jo1 阳性患者。回顾性收集了大型国际多中心 ASSD 患者队列中的临床特征和疾病表现簇，并进行了分析。
确定了 165 例（123 例女性）不完全 ASSD 患者。95 例（57.5%）患者在发病后 15 个月中位数（IQR 9-51）出现新的经典三联征表现，40 例（24%）患者在发病后 19 个月中位数（IQR 6-56）出现新的伴随特征。在随访期间，在出现新伴随表现的 40 例患者中的 32 例（80%）和未出现新伴随表现的 125 例患者中的 63 例（50.5%）中观察到三联征特征的新发，差异有统计学意义（p=0.002）。在至少出现一种新伴随特征的患者中，出现新三联征表现的几率比未出现新伴随发现的患者高 3.94 倍（95%CI 1.68-9.21，p=0.002）。
发病时表现为不完全形式的抗-Jo1 ASSD 患者随后出现缺乏经典三联征表现的风险较高。虽然所有 ASSD 患者均应仔细评估新三联征表现的发生，但对于新出现伴随表现的患者，应考虑更密切的随访。与未出现新伴随表现的患者相比，这些患者出现新的经典三联征表现的几率增加近 4 倍。
