• Satoshi Takanashi, Mitsuhiro Akiyama, Katsuya Suzuki, Kotaro Otomo, and Tsutomu Takeuchi.
• Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.
• Medicine (Baltimore). 2018 Jun 1; 97 (22): e10935.

RationaleImmunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory disease characterized by elevated serum IgG4 levels with infiltration of IgG4+ plasma cells and severe fibrosis in affected tissues. Recently, idiopathic fibrosing mediastinitis (FM), an extremely rare fibroinflammatory disorder, has been recognized as a form of IgG4-RD. As IgG4-RD can be treated by glucocorticoids, identification of the etiology of FM by surgical biopsy is essential; however, mediastinal biopsy is often difficult. We report 2 cases of IgG4-related FM successfully diagnosed with computed tomography (CT)-guided percutaneous needle biopsy.Patient ConcernsCase 1 was a 66-year-old woman with elevated serum C-reactive protein without any symptoms and case 2 was 78-year-old woman with abnormal mediastinal contour on chest x-ray. By further work-up, both cases were found to have mediastinitis accompanied by elevated serum IgG4. CT-guided percutaneous needle biopsy revealed massive infiltration of IgG4+plasma cells along with storiform fibrosis.DiagnosisIgG4-related FM.InterventionsGlucocorticoid therapy.OutcomeThe treatment resulted in significant improvement of the lesions after 3 months.LessonsEarly recognition and diagnosis of IgG4-related FM is essential because a delay in appropriate treatment initiation leads to progressive fibrosis with irreversible organ damage and poor prognosis. Our cases highlight CT-guided percutaneous needle biopsy as a promising option for histological examination in patients with IgG4-related FM.

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