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.
Highlights
IgG4-related disease (IgG4-RD) is recognized to include a growing number of fibroinflammatory disorders [1,2,3,4,5]
IgG4-RD was first described in the context of autoimmune pancreatitis presenting with obstructive jaundice due to a space-occupying lesion within the pancreas [7, 8]
The histopathology of the remaining cases was independently reviewed by two of the investigators (TVC and ESY) and a diagnosis of Fibrosing mediastinitis (FM) was confirmed in 15 patients
Summary
IgG4-related disease (IgG4-RD) is recognized to include a growing number of fibroinflammatory disorders [1,2,3,4,5]. IgG4-RD was first described in the context of autoimmune pancreatitis presenting with obstructive jaundice due to a space-occupying lesion within the pancreas [7, 8]. Since these initial reports, IgG4-RD has been demonstrated to involve various other organs including the biliary tree (sclerosing cholangitis), salivary (sclerosing sialadenitis), and lacrimal glands (sclerosing dacroadenitis) in isolation or in combination (multisystem involvement) [1,2,3,4,5, 9]. Patients with IgG4-RD typically respond to immunosuppressive therapy with glucocorticoids [11]
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