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A Case of Isolated IgG4-Related Interstitial PneumoniaA Case of IgG4-Related Interstitial Pneumonia: A New Consideration for the Cause of Idiopathic Nonspecific Interstitial Pneumonia

Kosuke Tanaka, MD; Kazuma Nagata, MD; Keisuke Tomii, MD, PhD; Yukihiro Imai, MD, PhD
Author and Funding Information

From the Department of Respiratory Medicine (Drs Tanaka, Nagata, and Tomii), and the Department of Clinical Pathology (Dr Imai), Kobe City Medical Center, General Hospital, Kobe, Japan.

Correspondence to: Kosuke Tanaka, MD, Department of Respiratory Medicine, Kobe City Medical Center, General Hospital, 2-1-1 Minatojima-minamimachi, Chuo-ku, Kobe, 650-0047, Japan; e-mail: kosuke624t@gmail.com

Financial/nonfinancial disclosures: The authors have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.


Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2012;142(1):228-230. doi:10.1378/chest.11-1858
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The 2008 American Thoracic Society Project concluded that idiopathic nonspecific interstitial pneumonia (NSIP), once regarded as a “provisional” diagnosis, can be considered a distinct clinical entity. However, an increasing number of recent reports have shown a likely link between the entity of idiopathic NSIP and autoimmune diseases. Similarly, IgG4-related disease is being increasingly reported, and some of the lung lesions, at least radiologically, look like NSIP. A case of IgG4-related interstitial lung disease without other systemic manifestations of IgG4-related disease is reported. The existence of IgG4-related disease should, therefore, be taken into consideration as a possible differential diagnosis from NSIP. The cause of IgG4-related disease remains unknown, but we emphasize the importance of a clinically novel interstitial lung disease.

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