Histopathologic overlap between fibrosing mediastinitis and IgG4-related disease

Tobias D Peikert, Bijayee Shrestha, Marie Christine Aubry, Thomas V. Colby, Jay H Ryu, Hiroshi Sekiguchi, Thomas Christopher Smyrk, Ulrich Specks, Eunhee S. Yi

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Abstract

Fibrosing mediastinitis (FM) and IgG4-related disease (IgG4-RD) are two fibroinflammatory disorders with potentially overlapping clinical and radiological features. In this paper, we looked for histopathologic features of IgG4-RD and enumerated infiltrating IgG4-positive plasma cells within mediastinal tissue biopsies from FM patients. We identified 15 consecutive FM surgical mediastinal tissue biopsies between 1985 and 2006. All patients satisfied the clinical and radiological diagnostic criteria for FM. All patients had either serological or radiological evidence of prior histoplasmosis or granulomatous disease, respectively. Formalin-fixed paraffin-embedded tissue sections of all patients were stained for H&E, IgG, and IgG4. Three samples met the predefined diagnostic criteria for IgG4-RD. In addition, characteristic histopathologic changes of IgG4-RD in the absence of diagnostic numbers of tissue infiltrating IgG4-positive plasma cells were seen in a number of additional cases (storiform cell-rich fibrosis in 11 cases, lymphoplasmacytic infiltrate in 7 cases, and obliterative phlebitis/arteritis in 2 cases). We conclude that up to one-third of histoplasmosis or granulomatous-disease- associated FM cases demonstrate histopathological features of IgG4-RD spectrum. Whether these changes occur as the host immune response against Histoplasma or represent a manifestation of IgG4-RD remains to be determined. Studies to prospectively identify these cases and evaluate their therapeutic responses to glucocorticoids and/or other immunosuppressive agents such as rituximab are warranted.

Original languageEnglish (US)
Article number207056
JournalInternational Journal of Rheumatology
Volume2012
DOIs
StatePublished - 2012

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Immunoglobulin G
Histoplasmosis
Plasma Cells
Mediastinal Fibrosis
Histoplasma
Biopsy
Phlebitis
Arteritis
Immunosuppressive Agents
Paraffin
Formaldehyde
Glucocorticoids
Fibrosis

ASJC Scopus subject areas

  • Rheumatology
  • Immunology

Cite this

Histopathologic overlap between fibrosing mediastinitis and IgG4-related disease. / Peikert, Tobias D; Shrestha, Bijayee; Aubry, Marie Christine; Colby, Thomas V.; Ryu, Jay H; Sekiguchi, Hiroshi; Smyrk, Thomas Christopher; Specks, Ulrich; Yi, Eunhee S.

In: International Journal of Rheumatology, Vol. 2012, 207056, 2012.

Research output: Contribution to journalArticle

Peikert, Tobias D ; Shrestha, Bijayee ; Aubry, Marie Christine ; Colby, Thomas V. ; Ryu, Jay H ; Sekiguchi, Hiroshi ; Smyrk, Thomas Christopher ; Specks, Ulrich ; Yi, Eunhee S. / Histopathologic overlap between fibrosing mediastinitis and IgG4-related disease. In: International Journal of Rheumatology. 2012 ; Vol. 2012.
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