TY - JOUR T1 - Respiratory lesions in IgG4-related disease: Classification using 2019 ACR/EULAR criteria JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00120-2022 SP - 00120-2022 AU - Masamichi Komatsu AU - Hiroshi Yamamoto AU - Shoko Matsui AU - Yasuhiro Terasaki AU - Akira Hebisawa AU - Tae Iwasawa AU - Takeshi Johkoh AU - Tomohisa Baba AU - Atsushi Miyamoto AU - Tomohiro Handa AU - Keisuke Tomii AU - Yuko Waseda AU - Masashi Bando AU - Haruyuki Ishii AU - Yasunari Miyazaki AU - Akihiko Yoshizawa AU - Tamiko Takemura AU - Yoshinori Kawabata AU - Masayuki Hanaoka AU - Takashi Ogura AU - the Tokyo Diffuse Lung Disease Study Group Y1 - 2022/01/01 UR - http://openres.ersjournals.com/content/early/2022/05/26/23120541.00120-2022.abstract N2 - In 2019, the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) proposed classification criteria for IgG4-related disease (IgG4-RD) [1, 2]. IgG4-RD can cause fibroinflammatory lesions characterised by infiltration of abundant IgG4-positive plasma cells with fibrosis and an elevated serum IgG4 concentration [3–5]. Approximately 35% of patients with IgG4-RD present with intrathoracic lesions, including those involving the mediastinal lymph nodes, bronchial walls, and peribronchovascular bundles [6–8]. To increase diagnostic sensitivity and specificity, we previously proposed diagnostic criteria for IgG4-related respiratory disease (IgG4-RRD) [9]. However, particularly for cases with isolated pulmonary lesions, the diagnosis of IgG4-RRD is sometimes difficult and requires multidisciplinary discussion (MDD) [10]. Additionally, we recently reported data for 16 patients with IgG4-positive interstitial pneumonia, which should be treated as a separate entity from conventional IgG4-RRD given the relative differences in disease behaviour and responses to glucocorticoid (GC) treatment [11].FootnotesThis manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.Conflict of interest: Dr. Komatsu has nothing to disclose.Conflict of interest: Dr. Yamamoto has nothing to disclose.Conflict of interest: Dr. Matsui has nothing to disclose.Conflict of interest: Dr. Terasaki has nothing to disclose.Conflict of interest: Dr. Hebisawa has nothing to disclose.Conflict of interest: Dr. Iwasawa has nothing to disclose.Conflict of interest: Dr. Johkoh has nothing to disclose.Conflict of interest: Dr. Baba has nothing to disclose.Conflict of interest: Dr. Miyamoto has nothing to disclose.Conflict of interest: Dr. Handa has nothing to disclose.Conflict of interest: Dr. Tomii has nothing to disclose.Conflict of interest: Dr. Waseda has nothing to disclose.Conflict of interest: Dr. Bando has nothing to disclose.Conflict of interest: Dr. Ishii has nothing to disclose.Conflict of interest: Dr. Miyazaki has nothing to disclose.Conflict of interest: Dr. Yoshizawa has nothing to disclose.Conflict of interest: Dr. Takemura has nothing to disclose.Conflict of interest: Dr. Kawabata has nothing to disclose.Conflict of interest: Dr. Hanaoka has nothing to disclose.Conflict of interest: Dr. Ogura has nothing to disclose. ER -