Pulmonary manifestations of immunoglobulin G4-related sclerosing disease

Jay H Ryu, H. Sekiguchi, E. S. Yi

Research output: Contribution to journalArticle

107 Citations (Scopus)

Abstract

Immunoglobulin (Ig)G4-related sclerosing disease (ISD) (also called IgG4-related systemic disease, IgG4-related disease or hyper-IgG4 disease) is a recently described systemic fibroinflammatory disease associated with elevated circulating levels of IgG4. Although initial descriptions of this disorder focused on its pancreatic presentation (autoimmune pancreatitis), it has become apparent that ISD is a systemic disease with many facets. The lesion of ISD is characterised by lymphoplasmacytic inflammation, fibrosis, phlebitis and increased numbers of IgG4-positive plasma cells. The disease can either be localised to one or two organs, or be present with diffuse multi-organ disease. Furthermore, lesions in different organs can present simultaneously or metachronously. In the thorax, lesions associated with ISD have been described in the lung parenchyma, airways and pleura, as well as the mediastinum. Data published to date suggest that ISD may account for a portion of various fibroinflammatory conditions of unknown cause encountered in the chest, including inflammatory pseudotumours, idiopathic interstitial pneumonias, fibrosing mediastinitis, inflammatory pleural lesions and, occasionally, airway disease. Some aspects of pulmonary manifestations attributed to ISD remain controversial and additional studies are needed to clarify the relationship along with the increasing relevance of this disorder to pulmonary medicine.

Original languageEnglish (US)
Pages (from-to)180-186
Number of pages7
JournalEuropean Respiratory Journal
Volume39
Issue number1
DOIs
StatePublished - Jan 1 2012

Fingerprint

Immunoglobulins
Lung
Immunoglobulin G
Thorax
Idiopathic Interstitial Pneumonias
Plasma Cell Granuloma
Phlebitis
Pulmonary Medicine
Pleura
Mediastinum
Plasma Cells
Pancreatitis
Fibrosis
Inflammation

Keywords

  • Immunoglobulin G4
  • Interstitial lung disease
  • Lymphadenopathy
  • Pulmonary inflammation

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Pulmonary manifestations of immunoglobulin G4-related sclerosing disease. / Ryu, Jay H; Sekiguchi, H.; Yi, E. S.

In: European Respiratory Journal, Vol. 39, No. 1, 01.01.2012, p. 180-186.

Research output: Contribution to journalArticle

Ryu, Jay H ; Sekiguchi, H. ; Yi, E. S. / Pulmonary manifestations of immunoglobulin G4-related sclerosing disease. In: European Respiratory Journal. 2012 ; Vol. 39, No. 1. pp. 180-186.
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