IgG4-related pleural disease with aortitis and submandibular glands involvement successfully treated with corticosteroid
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Rheumatology International https://doi.org/10.1007/s00296-020-04555-y
INTERNATIONAL
CASE BASED REVIEW
IgG4‑related pleural disease with aortitis and submandibular glands involvement successfully treated with corticosteroid: case‑based review Shota Okamoto1 · Hiroto Tsuboi1 · Ryota Sato1 · Mayu Terasaki1 · Toshihiko Terasaki1 · Hirofumi Toko1 · Masaru Shimizu1 · Fumika Honda1 · Mizuki Yagishita1 · Ayako Ohyama1 · Izumi Kurata1 · Saori Abe1 · Hiroyuki Takahashi1 · Atsumu Osada1 · Shinya Hagiwara1 · Yuya Kondo1 · Isao Matsumoto1 · Takayuki Sumida1 Received: 21 September 2019 / Accepted: 13 March 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract IgG4-related disease (IgG4-RD) is a fibro-inflammatory condition characterized by increased serum IgG4 level, infiltration of lymphocytes and IgG4-positive ( IgG4+) plasma cells and fibrosis. It can occur in almost all organs, commonly affecting the pancreas, biliary tract, salivary and lacrimal glands and kidneys. However, reports of IgG4-RD accompanied by pathologically confirmed, IgG4-related pleural disease are scarce. Here, we present a case of a 64-year-old man with suspected malignant pleural mesothelioma based on imaging findings but finally diagnosed with IgG4-RD (including pleuritis, periaortitis and bilateral submandibular gland enlargement) based on a high serum IgG4 level and pleural histopathological findings such as lymphoplasmacytic infiltration including IgG4+ plasma cells and fibrosis. Systemic corticosteroid therapy was effective at reducing serum IgG4, improving bilateral submandibular gland enlargement, and regressing pleural thickening and periaortic soft tissue. We also discuss clinical characteristics and pleural pathological features of previously reported cases with IgG4-related pleural disease based on a comprehensive literature review. Our case of IgG4-RD with pleura, aorta and submandibular gland involvement, pathologically confirmed by pleural specimen might be unique and very rare. Keywords IgG4-related disease · Pleural disease · Periaortitis · Submandibular gland
* Takayuki Sumida [email protected]
Ayako Ohyama [email protected]
Shota Okamoto [email protected]
Izumi Kurata [email protected]
Hiroto Tsuboi hiroto‑[email protected]
Saori Abe [email protected]
Ryota Sato [email protected]
Hiroyuki Takahashi [email protected]
Mayu Terasaki [email protected]
Atsumu Osada [email protected]
Toshihiko Terasaki [email protected]
Shinya Hagiwara [email protected]
Hirofumi Toko [email protected]
Yuya Kondo [email protected]
Masaru Shimizu [email protected]
Isao Matsumoto [email protected]
Fumika Honda [email protected]
1
Mizuki Yagishita [email protected]
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, 1‑1‑1 Tennodai, Tsukuba, Ibaraki 305‑8575, Japan
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Introduction IgG4-related disease (IgG4-RD) is a syst
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