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

4 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

Fingerprint

Needle Biopsy
Immunoglobulin G
Tomography
Immunoglobulins
Fibrosis
Plasma Cells
Glucocorticoids
Biopsy
Mediastinitis
Mediastinal Fibrosis
Serum
C-Reactive Protein
Blood Proteins
Early Diagnosis
Thorax
X-Rays
Therapeutics

Keywords

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

ASJC Scopus subject areas

  • Medicine(all)

Cite this

IgG4-related fibrosing mediastinitis diagnosed with computed tomography-guided percutaneous needle biopsy. / Takanashi, Satoshi; Akiyama, Mitsuhiro; Suzuki, Katsuya; Otomo, Kotaro; Takeuchi, Tsutomu.

In: Medicine (United States), Vol. 97, No. 22, e10935, 01.06.2018.

Research output: Contribution to journalArticle

@article{cb9a95bef1544850a7ce24ec6cd5d8fd,
title = "IgG4-related fibrosing mediastinitis diagnosed with computed tomography-guided percutaneous needle biopsy",
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.",
keywords = "computed tomography guided percutaneous needle biopsy, fibrosing mediastinitis, IgG4-related disease, M2 macrophage, storiform fibrosis",
author = "Satoshi Takanashi and Mitsuhiro Akiyama and Katsuya Suzuki and Kotaro Otomo and Tsutomu Takeuchi",
year = "2018",
month = "6",
day = "1",
doi = "10.1097/MD.0000000000010935",
language = "English",
volume = "97",
journal = "Medicine; analytical reviews of general medicine, neurology, psychiatry, dermatology, and pediatries",
issn = "0025-7974",
publisher = "Lippincott Williams and Wilkins",
number = "22",

}

TY - JOUR

T1 - IgG4-related fibrosing mediastinitis diagnosed with computed tomography-guided percutaneous needle biopsy

AU - Takanashi, Satoshi

AU - Akiyama, Mitsuhiro

AU - Suzuki, Katsuya

AU - Otomo, Kotaro

AU - Takeuchi, Tsutomu

PY - 2018/6/1

Y1 - 2018/6/1

N2 - 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.

AB - 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.

KW - computed tomography guided percutaneous needle biopsy

KW - fibrosing mediastinitis

KW - IgG4-related disease

KW - M2 macrophage

KW - storiform fibrosis

UR - http://www.scopus.com/inward/record.url?scp=85048312008&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85048312008&partnerID=8YFLogxK

U2 - 10.1097/MD.0000000000010935

DO - 10.1097/MD.0000000000010935

M3 - Article

VL - 97

JO - Medicine; analytical reviews of general medicine, neurology, psychiatry, dermatology, and pediatries

JF - Medicine; analytical reviews of general medicine, neurology, psychiatry, dermatology, and pediatries

SN - 0025-7974

IS - 22

M1 - e10935

ER -