Immunoglobulin G4-related thoracic disease: clinical and radiological findings of a Turkish cohort
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Keywords
IgG4-related disease, Lung, Thorax, Airway, Pleura, Mediastinum
Abstract
Background and Aim: Thoracic involvement of Immunoglobulin G4-related disease (IgG4-RD) is relatively rare and may be disregarded at the time of initial diagnosis due to its asymptomatic nature. This study aimed to ascertain the prevalence and patterns of thoracic involvement in a retrospective cohort of Turkish patients with IgG4-RD. Methods: A total of 90 patients (47 males and 43 females, with a mean age of 57.7±15.5 years) diagnosed with IgG4-RD were reviewed retrospectively. All computed tomography (CT) scans were re-evaluated by two thoracic radiologists and IgG4-related thoracic disease was assessed on four compartments: The mediastinum, pulmonary parenchyma, airways, and pleura. IgG4-related thoracic disease was categorized as: definite, highly probable, probable or possible. Results: There were 64 patients who had undergone at least one thorax CT examination, and 18 (28%) were diagnosed with IgG4-related thoracic disease. The rate of IgG4-related thoracic disease increased by 20% and reached a ratio of 48.4% (n=31) after a thorough reevaluation of registry data specifically to thoracic findings. The mediastinum was the most frequently involved compartment, affecting 16 (51.6%) patients, followed by pulmonary parenchyma in 14 (45.2%) patients, and airways and pleura in 10 (32.3%) patients each. Other organ involvements were more prevalent and IgG4 levels were higher in patients with thoracic involvement. Eosinophils were significantly elevated in patients with thoracic involvement (p=0.023). Conclusions: IgG4-related thoracic disease is heterogeneous and likely to be more prevalent than currently recognized. The mediastinum is the most frequently involved compartment. It is important to assess IgG4-related thoracic disease at the time of initial diagnosis. Elevated levels of serum IgG4 and eosinophils, as well as a greater number of organ involvements may serve as indicators of thoracic involvement.
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Aslı Alkan, Yildirim Beyazit University, Yenimahalle Training and Research Hospital, Chest Diseases Clinic, Ankara, Turkey
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Gamze Durhan, Hacettepe University Faculty of Medicine, Department of Radiology, Ankara, Turkey
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Gozde Kubra Yardimci, Hacettepe University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Hacettepe University Vasculitis Research Centre, Ankara, Turkey
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Umran Ozden Sertcelik, Ankara City Hospital, Chest Diseases Clinic, Ankara, Turkey
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Bayram Farisogullari, Hacettepe University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Hacettepe University Vasculitis Research Centre, Ankara, Turkey
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Macit Ariyurek, Hacettepe University Faculty of Medicine, Department of Radiology, Ankara, Turkey
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Omer Karadag, Hacettepe University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Hacettepe University Vasculitis Research Centre, Ankara, Turkey
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Deniz Koksal, Hacettepe University Faculty of Medicine, Department of Chest Diseases, Ankara, Turkey
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