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

• Published in: Clinical rheumatology Vol. 42; no. 5; pp. 1363 - 1370
• Main Authors: Şen, Nesrin, Acer Kasman, Sevtap, Baysal, Tamer, Dizman, Rıdvan, Yılmaz-Öner, Sibel, Tezcan, Mehmet Engin
• Format: Journal Article
• Language: English
• Published: Cham Springer International Publishing 01.05.2023; Springer Nature B.V
• Subjects: Comorbidity; Computed tomography; Demography; Disease; Familial Mediterranean fever; Familial Mediterranean Fever - genetics; Fever; Fibrosis; Humans; Inflammatory diseases; Lung - diagnostic imaging; Lung diseases; Medicine; Medicine & Public Health; Original; Original Article; Pleurisy; Rheumatic diseases; Rheumatology; Spondyloarthropathy; Thorax; Familial Mediterranean fever; Thorax; Thorax computerized tomography; Apical fibrosis
• ISSN: 0770-3198; 1434-9949
• DOI: 10.1007/s10067-023-06526-7

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 .