Ruscitti Piero, Berardicurti Onorina, Iacono Daniela, Pantano Ilenia, Liakouli Vasiliki, Caso Francesco, Emmi Giacomo, Grembiale Rosa Daniela, Cantatore Francesco Paolo, Atzeni Fabiola, Perosa Federico, Scarpa Raffaele, Guggino Giuliana, Ciccia Francesco, Barile Antonio, Cipriani Paola, Giacomelli Roberto
Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, delta 6 building, PO box 67100, L'Aquila, Italy.
Rheumatology Section, Department of Precision Medicine, University of Campania "Luigi Vanvitelli", Naples, Italy.
Arthritis Res Ther. 2020 Jun 22;22(1):151. doi: 10.1186/s13075-020-02245-5.
Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown aetiology usually affecting young adults. Interestingly, recent evidence from the juvenile counterpart of AOSD suggested the emergent high fatality rate of lung disease (LD) in these patients. In this work, we aimed to characterise LD in AOSD, to identify associated clinical features and predictive factors, and to describe long-term outcomes of the disease comparing patients with LD and those without.
A retrospective assessment of prospectively followed patients, from January 2001 to December 2019, was provided to describe the rate of LD in AOSD, associated clinical features and predictive factors, and long-term outcomes. Patients with AOSD, who were included in Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale (GIRRCS) cohort, were assessed.
Out of 147 patients included in GIRRCS cohort, 18 (12.25%) patients were reported to be affected by LD, at the time of diagnosis of AOSD, who were characterised by older age, a higher prevalence of myalgia, of lymph node involvement, of pleuritis, and abdominal pain. Furthermore, patients with LD showed higher values of systemic score and ferritin. Among those clinical variables, older age and systemic score were also independently predictors of LD. Chest CT scans were also obtained, and the most common finding was the peripheral consolidations in 8 (44.4%) patients. Finally, a higher mortality rate, of 38.9%, was registered in patients with LD than others, since it was associated with a significant decreased survival rate.
The presence of LD could suggest an emergent cause of mortality in AOSD, as observed in juvenile counterpart recognising a further marker of severity and poor prognosis to be careful evaluated. Patients with LD were also characterised by some clinical features, higher values of systemic score and ferritin than the others, identifying a subset of patients mostly burdened by systemic signs and symptoms. Although specific designed future studies are needed to fully elucidate the significance of LD in AOSD, a more accurate evaluation and management of this feature could improve the long-term outcomes of these patients.
成人斯蒂尔病（AOSD）是一种病因不明的全身性炎症性疾病，通常影响年轻人。有趣的是，最近来自AOSD青少年患者的证据表明，这些患者中肺部疾病（LD）的死亡率急剧上升。在这项研究中，我们旨在描述AOSD中的LD特征，确定相关的临床特征和预测因素，并比较有LD和无LD患者的疾病长期结局。
对2001年1月至2019年12月前瞻性随访的患者进行回顾性评估，以描述AOSD中LD的发生率、相关临床特征和预测因素以及长期结局。对纳入意大利临床与实验风湿病研究组（GIRRCS）队列的AOSD患者进行评估。
在GIRRCS队列纳入的147例患者中，18例（12.25%）在AOSD诊断时被报告患有LD，其特征为年龄较大、肌痛、淋巴结受累、胸膜炎和腹痛的患病率较高。此外，LD患者的全身评分和铁蛋白值更高。在这些临床变量中，年龄较大和全身评分也是LD的独立预测因素。还进行了胸部CT扫描，最常见的发现是8例（44.4%）患者出现外周实变。最后，LD患者的死亡率较高，为38.9%，因为这与生存率显著降低相关。
LD的存在可能提示AOSD中一个新的死亡原因，正如在青少年患者中观察到的那样，这是一个需要仔细评估的严重程度和预后不良的进一步标志。LD患者还具有一些临床特征，全身评分和铁蛋白值高于其他患者，这确定了一组主要受全身症状和体征困扰的患者亚组。尽管需要未来进行专门设计的研究来充分阐明LD在AOSD中的意义，但对这一特征进行更准确的评估和管理可能会改善这些患者的长期结局。
