Department of Rheumatology, Kartal Dr. Lutfi Kirdar City Hospital, Kartal, 34680, Istanbul, Turkey.
Department of Radiology, Kartal Dr. Lutfi Kirdar City Hospital, Istanbul, Turkey.
Clin Rheumatol. 2023 May;42(5):1363-1370. doi: 10.1007/s10067-023-06526-7. Epub 2023 Feb 1.
Familial Mediterranean fever (FMF) is one of the common autoinflammatory diseases with multisystemic manifestation. Pleuritis is the only known pulmonary involvement of FMF; however, as far as we know, thoracic involvements in pleural, parenchymal, bronchial, and vascular structures have not been evaluated yet.
We included 243 consecutive FMF patients who applied to our clinic within the last 5 years and were requested to have a thorax CT for any reason and 122 trauma patients without any comorbidity. An experienced radiologist evaluated the thorax CT images blindly according to the relevant guidelines. We then presented the common incidental pulmonary and mediastinal findings on the thorax CT. Additionally, we compared patients with and without lung involvement according to demographic and disease-related parameters.
In our study, 167 of 243 patients (68.7%) had at least one of the pulmonary findings on their thorax CT. The most common pulmonary findings were apical fibrosis in 96 (39.5%) patients, parenchymal fibrotic changes in 48 (19.8%) patients, and a solitary parenchymal nodule smaller than 4 mm in 33 (13.6%) patients. All demographic, genetic, and disease-related characteristics, including the frequency of spondyloarthropathy, were similar in patients with and without pulmonary findings.
We showed that the most common incidental pulmonary finding in our FMF cohort was apical fibrosis on thoracic CT. Our data did not show causality between FMF and apical fibrosis; therefore, more studies are needed to evaluate the frequency and clinical significance of apical fibrosis in FMF. Key Points • More than two-thirds of familial Mediterranean fever (FMF) patients in our study group who underwent a thoracic scan for any reason had pulmonary and mediastinal findings on thorax computed tomography (CT). • In our FMF cohort, the most common incidental pulmonary finding on their thorax CT was apical fibrosis. • All demographic and disease-related characteristics, including the frequency of spondyloarthritis, were similar between patients with and without pulmonary and mediastinal findings.
家族性地中海热（FMF）是一种常见的多系统自身炎症性疾病。胸膜炎是 FMF 已知的唯一肺部受累表现；然而，据我们所知，尚未评估胸膜、实质、支气管和血管结构的胸部受累情况。
我们纳入了 243 名在过去 5 年内因任何原因就诊于我们诊所并要求进行胸部 CT 的连续 FMF 患者，以及 122 名无合并症的创伤患者。一位有经验的放射科医生根据相关指南对胸部 CT 图像进行了盲法评估。然后，我们提出了胸部 CT 上常见的偶然肺部和纵隔表现。此外，我们根据人口统计学和疾病相关参数比较了有和无肺部受累的患者。
在我们的研究中，243 名患者中有 167 名（68.7%）在其胸部 CT 上至少有一项肺部发现。最常见的肺部发现是 96 名患者（39.5%）的尖部纤维化、48 名患者（19.8%）的实质纤维性改变和 33 名患者（13.6%）的小于 4 毫米的孤立实质结节。所有人口统计学、遗传和疾病相关特征，包括脊柱关节病的频率，在有和无肺部发现的患者中均相似。
我们表明，我们的 FMF 队列中最常见的偶然肺部发现是胸部 CT 上的尖部纤维化。我们的数据并未显示 FMF 和尖部纤维化之间存在因果关系；因此，需要更多的研究来评估 FMF 中尖部纤维化的频率和临床意义。关键点：
在我们因任何原因接受胸部扫描的研究组中，超过三分之二的家族性地中海热（FMF）患者在胸部 CT 上有肺部和纵隔表现。
在我们的 FMF 队列中，胸部 CT 上最常见的偶然肺部发现是尖部纤维化。
所有人口统计学和疾病相关特征，包括脊柱关节炎的频率，在有和无肺部和纵隔发现的患者之间相似。
