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We previously reported respiratory involvement in 25 patients with autoimmune pancreatitis, a pancreatic manifestation of IgG4-related disease that responds well to glucocorticoid treatment. However, whether all respiratory lesions in patients with autoimmune pancreatitis have genuine respiratory involvement is unclear. This study aimed to update respiratory lesions' clinical and radiological characteristics in patients with autoimmune pancreatitis. We retrospectively reviewed the clinical and radiological data of 74 consecutive patients diagnosed with autoimmune pancreatitis at Shinshu University Hospital and treated with glucocorticoid. Clinical features and chest high-resolution computed tomography findings before and after therapy were reviewed. Fifty-one patients (68.9%) had respiratory lesions. In 65 of the 74 patients, chest high-resolution computed tomography results were evaluated before and after treatment. Patients with IgG4-related disease and respiratory lesions showed significantly higher serum IgG4 levels and hypocomplementemia than those without respiratory lesions; they also had more affected organs. While most abnormal thoracic findings improved, 4 cases of 7 with reticular opacities and all 11 cases with emphysema did not improve. Therefore, these lesions with poor response to glucocorticoid treatment should not be considered due to respiratory involvement of autoimmune pancreatitis based on the current classification criteria for IgG4-related disease. Patients with autoimmune pancreatitis and respiratory lesions exhibited higher disease activity than those without. Most chest high-resolution computed tomography lesions were responsive to glucocorticoid treatment, whereas reticular opacities and emphysema were poorly responsive.
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