TY - JOUR T1 - Cytokine profiles in the BAL fluid of IgG<sub>4</sub>-related respiratory disease compared with sarcoidosis JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00009-2015 VL - 1 IS - 2 SP - 00009-2015 AU - Hiroshi Yamamoto AU - Masanori Yasuo AU - Takashi Ichiyama AU - Atsuhito Ushiki AU - Masayuki Hanaoka AU - Hideaki Hamano AU - Takeshi Uehara AU - Satoshi Kawakami AU - Shoko Matsui AU - Keishi Kubo Y1 - 2015/10/01 UR - http://openres.ersjournals.com/content/1/2/00009-2015.abstract N2 - IgG4-related disease (IgG4-RD) is a multiorgan disorder that involves the salivary glands, pancreas and lungs [1]. We previously reported six patients with autoimmune pancreatitis (AIP) who showed characteristic central airway stenosis and bilateral hilar lymphadenopathy (BHL) that mimics sarcoidosis [2, 3]. We subsequently prospectively identified four additional patients with AIP and one patient with IgG4-related kidney disease (IgG4-RKD) with similar chest computed tomography (CT) findings and IgG4-positive plasma cell infiltration without granuloma in the airways. 10 out of 11 patients were diagnosed with AIP by their gastroenterologists based on the diagnostic AIP criteria proposed by the Japanese Pancreatic Society in 2011 [4] and the remaining one patient was diagnosed with IgG4-RKD by renal physicians based on the renal biopsy specimens in our hospital. All the 11 patients showed BHL and bronchial wall thickening on chest CT and were underwent transbronchial lung biopsy and bronchial biopsy according to our routine protocol [3]. The median IgG4-positive cell count and the percentage of IgG4-positive cells to IgG-positive plasma cells was 54.3 (range 17–80.6) per high-power field and 63.6% (range 34.5–96.5%), respectively. Thus, we diagnosed the 11 patients with IgG4-related respiratory disease (IgG4-RRD) characterised by airway involvement and BHL at multidisciplinary meetings of our hospital, as previously reported [3]. None of the 11 patients had oxygen desaturation and seven out of 11 patients had cough. The 10 patients with AIP showed one or more extrapulmonary manifestations other than in the pancreas and the one patient with IgG4-RKD had submandibular involvement. Nine patients with AIP received oral corticosteroid therapy for pancreatic lesions, whereas one patient did not because of the presence of severe cataract and glaucoma. The patient with IgG4-RKD did not receive oral corticosteroid therapy because spontaneous improvement of renal function was observed. Two patients received inhaled steroid therapy for cough. All 11 patients were alive at the end of our study and had no respiratory symptoms from these therapies.BAL cytokines of IgG4-RRD patients are more characteristic of the Th2 response than those of sarcoidosis patients http://ow.ly/T2gDVWe thank Shigeyuki Kawa (Center for Health, Safety and Environmental Management, Shinshu University School of Medicine, Matsumoto, Japan) for expert advice and management of some of the IgG4-RD patients. We thank Hitomi Imamura (First Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan) and Akane Sueki (Department of Laboratory Medicine, Shinshu University School of Medicine, Matsumoto, Japan) for skilled technical assistance with the cytokine measurements, and Yunden Droma (First Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan) for help in manuscript preparation. ER -