Long-term clinical course and outcomes of immunoglobulin G4-related lung disease
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RESEARCH
Long‑term clinical course and outcomes of immunoglobulin G4‑related lung disease Jieun Kang1,2†, Shinhee Park3†, Eun Jin Chae4, Joon Seon Song5, Hee Sang Hwang5, Sun Jong Kim6, Tae Jun Song7, Myung‑Whan Kim7 and Jin Woo Song1*
Abstract Background: Immunoglobulin G4-related lung disease (IgG4-RLD) is the pulmonary manifestation of a systemic fibroinflammatory disease characterized by lymphoplasmacytic infiltration with an abundance of IgG4-positive plasma cells. Long-term clinical course and outcomes of IgG4-RLD remain unclear. We aimed to identify clinical characteristics, treatment outcomes, and longitudinal pulmonary function changes in patients with IgG4-RLD according to the radiologic classification. Methods: Chest computed tomography findings of 37 subjects were classified into five subtypes: solid nodular, bronchovascular, alveolar interstitial, round ground glass opacity, and alveolar consolidative. Radiologic treatment outcomes and longitudinal pulmonary function changes were compared among the different radiologic subtypes. Results: The mean age of the subjects was 55.6 years, and 78.4% were male. Among the five radiologic subtypes, alveolar consolidative and solid nodular type were most common, accounting for approximately 29.7% each of the total cases. Prednisone with or without azathioprine was administered to 31 patients (median treatment duration 14 months). In the treated patients, serial images showed complete response or partial response in 77.4%. However, relapse was documented in 25.0% of those who showed complete or partial response. In patients whose longitudinal lung function data were available (n = 20), the lung function was found to be stable during follow-up. Alveolar consolidative type showed the highest complete response rate, whereas alveolar interstitial type showed the lowest response rate, either complete or partial. Conclusions: Most patients showed a favorable outcome with regards to radiologic improvement and maintenance of pulmonary function; however, the response differed according to the radiologic subtype. Keywords: Immunoglobulin G4-related disease, Treatment outcome, Lung function, Chest computed tomography Background Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized systemic fibroinflammatory disease characterized by lymphoplasmacytic infiltration with an abundance of IgG4-positive plasma cells [1]. *Correspondence: [email protected] † Jieun Kang and Shinhee Park contributed equally to this work 1 Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic‑Ro 43‑gil, Songpa‑gu, Seoul 05505, Republic of Korea Full list of author information is available at the end of the article
It can affect various organs including the pancreas, hepatobiliary system, kidney, retroperitoneum, and lacrimal and salivary glands [2]. Lung involvement can either be the sole manifestation or part of multiple organ involvements [3]. IgG4-related lung disease (IgG4-RLD) has hete
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