Apical fibrosis was the most common incidental pulmonary finding in a familial Mediterranean fever cohort

Abstract

Introduction 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. Method 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. Results 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. Conclusions 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 .

Is apical lung fibrosis really associated with familial Mediterranean fever?

“…We read with great surprise the study conducted by Nesrin Şen et al [1]. Many methodological errors in this study need to be pointed out.…”

Authors’ response: Main article "Apical fibrosis was the most common incidental pulmonary finding in a familial Mediterranean fever cohort"

“…Second, neither pulmonary fibrosis nor apical fibrosis can be linked to only SpA according to this study's results. The primary goal of our study [1] is to highlight the most prevalent incidental pulmonary computerized tomography (CT) findings in adult individuals with familial Mediterranean fever (FMF). When we discovered apical fibrosis as the most common thorax CT sign, we needed to differentiate patients with SpA because apical fibrosis is commonly seen in SpA.…”

“…As there is no other research in this field, it provides simple findings and creates curiosity. In the study [1], a radiologist with over 20 years of experience (professor of radiology) analysed the participants' thorax CTs in accordance with the guidelines. It would provide more reliable results if we performed inter-and intra-observer analysis.…”