IgG4-related fibrosing mediastinitis diagnosed with computed tomography-guided percutaneous needle biopsy

Satoshi Takanashi, Mitsuhiro Akiyama, Katsuya Suzuki, Kotaro Otomo, Tsutomu Takeuchi

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

Rationale: Immunoglobulin 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 concerns: Case 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. Diagnosis: IgG4-related FM. Interventions: Glucocorticoid therapy. Outcome: The treatment resulted in significant improvement of the lesions after 3 months. Lessons: Early 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.

Original languageEnglish
Article numbere10935
JournalMedicine (United States)
Volume97
Issue number22
DOIs
Publication statusPublished - 2018 Jun 1

Keywords

  • computed tomography guided percutaneous needle biopsy
  • fibrosing mediastinitis
  • IgG4-related disease
  • M2 macrophage
  • storiform fibrosis

ASJC Scopus subject areas

  • Medicine(all)

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