Pulmonary Findings in Children With Colchicine-Resistant Familial Mediterranean Fever.

Background: Approximately 5%-10% of Familial Mediterranean Fever (FMF) patients fail to achieve full disease control despite adequate colchicine therapy and these patients may be vulnerable for lung involvement.

Objective: This study aimed to examine pulmonary findings on thoracic computerized tomography (CT) in children with colchicine-resistant FMF (crFMF), and their correlation with clinical characteristics regarding pulmonary involvement in childhood FMF is not well understood, and there is a lack of research specifically evaluating crFMF cases.

Methods: This cross-sectional study investigated pulmonary findings in 31 patients diagnosed with crFMF, focusing on thoracic CT scan findings and respiratory symptoms such as dry cough, shortness of breath, and exercise intolerance. The study further incorporated spirometry evaluations to assess pulmonary functions.

Results: Of the patients, 29.1% had normal thoracic CT scans, whereas 70.9% showed abnormalities, with nodules being the most common finding (61.2%). One patient demonstrated subpleural honeycombing and bilateral ground-glass opacities, indicating FMF-related interstitial lung disease (ILD). Chest pain was the predominant symptom during the attacks (70.9%), while shortness of breath persisted most frequently. While 32% of the patients had abnormal values of spirometry at postattack period, the rate was 14.2% when they were clinically stable. There was an increase at mean forced expiratory flow at 25%-75% of FVC (FEF25-75) at the stable phase. (83.1 ± 19.2 vs. 92.2 ± 21.6, p = 0.05). No significant differences in CT findings were noted between genotypes and ISSF scores.

Conclusions: The study highlights a high prevalence of radiological findings in patients with crFMF; notably, a case with findings suggestive of FMF-related interstitial lung involvement was defined. Respiratory symptoms and abnormal values at spirometry were frequently accompanied by FMF attacks. These findings underscore the need for respiratory evaluation thoroughly in patients with crFMF to detect subclinical or overt pulmonary involvement and to guide appropriate management strategies.