Short Communication
Proposed diagnostic criteria for IgG4-related respiratory disease

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Background on the preparation of the diagnostic criteria

IgG4-related disease (IgG4-RD) [1] is a newly recognized systemic disease characterized by an elevated serum level of IgG4, infiltration of IgG4-positive plasma cells into affected lesions, and fibrosis. In 2011, comprehensive diagnostic criteria for IgG4-RD were proposed by the Study Group of Intractable Diseases from the Ministry of Health, Labor and Welfare, Japan (MHLW) [2], and these criteria have been gaining increasing acceptance.

The comprehensive diagnostic criteria [2] are highly

Nomenclature of respiratory tract lesions

At the International Symposium on IgG4-RD held in Boston in 2011, two separate names were adopted for the respiratory tract lesions of IgG4-related disease: ‘IgG4-related lung disease’ and ‘IgG4-related pleural disease’ [5]. However, since a subsequent report that respiratory tract lesions are typically interstitial lesions [6], the Ministry of Health group decided to refer to lesions of both the intrathoracic respiratory organs and auxiliary structures collectively as ‘IgG4-related respiratory

Summary

The diagnostic criteria of IgG4-RRD are outlined. We hope for further consideration and acceptance of the criteria.

Conflict of interest

None of the authors have any relevant conflicts of interest to report.

Acknowledgments

The authors would like to express their appreciation to Dr. Tomohiro Handa of Kyoto University for his valuable assistance in the preparation of this manuscript. They also gratefully acknowledge the financial support provided for this work by the research program for the Study of Intractable Diseases of the Ministry of Health, Labor, and Welfare of Japan.

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There are more references available in the full text version of this article.

Cited by (0)

This paper was previously peer-reviewed and published by the Journal of the Japanese Respiratory Society [7].

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