Abstract

BackgroundThe diagnosis of Immunoglobulin G4 (IgG4)-related disease (IgG4-RD), in general, depends on serum IgG4 concentrations and histopathological findings; therefore, diagnosis of IgG4-RD in cardiovascular organs/tissues is often difficult owing to the risk of tissue sampling.MethodsPrevalence of IgG4-positive lymphoplasmacytic infiltration in 103 consecutive cardiovascular surgical samples from 98 patients with various cardiovascular diseases was analyzed immunohistochemically.ResultsThe diagnoses of the enrolled patients included aortic aneurysm (abdominal, n = 8; thoracic, n = 9); aortic dissection (n = 20); aortic stenosis (n = 24), aortic regurgitation (n = 10), and mitral stenosis/regurgitation (n = 17). In total, 10 (9.7%) of the 103 specimens showed IgG4-positive cell infiltration with various intensities; five of these were aortic valve specimens from aortic stenosis, and IgG4-positive cell infiltration was present at >10 /HPF in three of them. In one aortic wall sample from an abdominal aortic aneurysm, various histopathological features of IgG4-RD, such as IgG4-positive cell infiltration, obliterating phlebitis, and storiform fibrosis, were observed.ConclusionsIgG4-positive cell infiltration was observed in 9.7% of the surgical cardiovascular specimens, mainly in the aortic valve from aortic stenosis and in the aortic wall from aortic aneurysm. Whether IgG4-positive cell infiltration has pathophysiological importance in the development or progression of cardiovascular diseases should be investigated in future studies.

Highlights

  • The diagnosis of Immunoglobulin G4 (IgG4)-related disease (IgG4-RD), in general, depends on serum IgG4 concentrations and histopathological findings; diagnosis of IgG4related disease (IgG4-RD) in cardiovascular organs/tissues is often difficult owing to the risk of tissue sampling

  • We have investigated the Hourai et al BMC Cardiovascular Disorders (2017) 17:52 prevalence and the extent of IgG4-positive lymphoplasmacytic infiltration observed in tissue samples of various cardiovascular diseases obtained at the time of surgery

  • IgG4positive cell infiltration was most frequently observed in aortic wall of the Abdominal aortic aneurysm (AAA) (3/8, 38%) followed by the aortic valve of aortic stenosis (5/24, 21%), and the aortic wall of dissecting aneurysm (2/20, 10%)

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Summary

Introduction

The diagnosis of Immunoglobulin G4 (IgG4)-related disease (IgG4-RD), in general, depends on serum IgG4 concentrations and histopathological findings; diagnosis of IgG4-RD in cardiovascular organs/tissues is often difficult owing to the risk of tissue sampling. But not all, cases of IgG4-RD in cardiovascular organs have diagnosed on the basis of histopathological findings in samples obtained either at the time of surgery or by autopsy. Biopsy of these vital organs in other situations is generally associated with considerable risk; IgG4-RD in cardiovascular organs may be underdiagnosed. We have investigated the Hourai et al BMC Cardiovascular Disorders (2017) 17:52 prevalence and the extent of IgG4-positive lymphoplasmacytic infiltration observed in tissue samples of various cardiovascular diseases obtained at the time of surgery

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Conclusion

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