Abstract
Pulmonary involvement in IgG4-related disease can manifest in any intrathoracic compartment including the lung parenchyma, airways, lymph nodes, mediastinum, and pleura. Although most patients with IgG4-related lung disease have extrapulmonary manifestations, some patients present with isolated pulmonary disease and may be difficult to diagnose. Approximately one-half of those with IgG4-related lung disease have respiratory symptoms at presentation. The most common form of intrathoracic involvement is mediastinal and/or hilar lymphadenopathy. Parenchymal manifestations of IgG4-related lung disease include single or multiple rounded opacities and interstitial lung disease. Airway disease can cause airway narrowing and asthma-like symptoms. Aside from mediastinal lymphadenopathy, IgG4-related fibrosing mediastinitis has also been reported. Pleural manifestations include pleural masses and pleural effusions. These patterns of intrathoracic disease are best characterized by CT (including high-resolution images of the lung parenchyma). Diagnostic confirmation of IgG4-related lung disease may require bronchoscopic or surgical biopsy, partly depending on the site of involvement. Histopathologic findings associated with IgG4-related lung disease are similar to those seen in extrapulmonary organs, but characteristic storiform fibrosis is not as apparent in lung biopsies. IgG4-related lung disease generally responds well to corticosteroid therapy.
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Zen Y, Nakanuma Y. IgG4-related disease: a cross-sectional study of 114 cases. Am J Surg Pathol. 2010;34(12):1812–19.
Tsushima K, Tanabe T, Yamamoto H, et al. Pulmonary involvement of autoimmune pancreatitis. Eur J Clin Invest. 2009;39(8):714–22.
Shrestha B, Sekiguchi H, Colby TV, et al. Distinctive pulmonary histopathology with increased IgG4-positive plasma cells in patients with autoimmune pancreatitis: report of 6 and 12 cases with similar histopathology. Am J Surg Pathol. 2009;33(10):1450–62.
Zen Y, Inoue D, Kitao A, et al. IgG4-related lung and pleural disease: a clinicopathologic study of 21 cases. Am J Surg Pathol. 2009;33(12):1886–93.
Shigemitsu H, Koss MN. IgG4-related interstitial lung disease: a new and evolving concept. Curr Opin Pulm Med. 2009;15(5):513–16.
Inoue D, Zen Y, Abo H, et al. Immunoglobulin G4-related lung disease: CT findings with pathologic correlations. Radiology. 2009;251(1):260–70.
Khosroshahi A, Stone JH. A clinical overview of IgG4-related systemic disease. Curr Opin Rheumatol. 2011;23(1):57–66.
Yamashita K, Haga H, Kobashi Y, Miyagawa-Hayashino A, Yoshizawa A, Manabe T. Lung involvement in IgG4-related lymphoplasmacytic vasculitis and interstitial fibrosis: report of 3 cases and review of the literature. Am J Surg Pathol. 2008;32(11):1620–6.
Zen Y, Kitagawa S, Minato H, et al. IgG4-positive plasma cells in inflammatory pseudotumor (plasma cell granuloma) of the lung. Hum Pathol. 2005;36(7):710–17.
Taniguchi T, Ko M, Seko S, et al. Interstitial pneumonia associated with autoimmune pancreatitis [comment]. Gut. 2004;53(5):770; author reply 770–1.
Kobayashi H, Shimokawaji T, Kanoh S, Motoyoshi K, Aida S. IgG4-positive pulmonary disease. J Thorac Imaging. 2007;22(4):360–2.
Hirano K, Kawabe T, Komatsu Y, et al. High-rate pulmonary involvement in autoimmune pancreatitis. Intern Med J. 2006;36(1):58–61.
Taniguchi T, Hamasaki A, Okamoto M. A case of suspected lymphocytic hypophysitis and organizing pneumonia during maintenance therapy for autoimmune pancreatitis associated with autoimmune thrombocytopenia. Endocr J. 2006;53(4):563–6.
Ohara H, Nakazawa T, Sano H, et al. Systemic extrapancreatic lesions associated with autoimmune pancreatitis. Pancreas. 2005;31(3):232–7.
Duvic C, Desrame J, et al. Retroperitoneal fibrosis, sclerosing pancreatitis and bronchiolitis obliterans with organizing pneumonia. Nephrol Dial Transplant. 2004;19(9):2397–9.
Ito M, Yasuo M, Yamamoto H, et al. Central airway stenosis in a patient with autoimmune pancreatitis. Eur Respir J. 2009;33(3):680–3.
Inoue M, Nose N, Nishikawa H, Takahashi M, Zen Y, Kawaguchi M. Successful treatment of sclerosing mediastinitis with a high serum IgG4 level. Gen Thorac Cardiovasc Surg. 2007;55(10):431–3.
Naitoh I, Nakazawa T, Ohara H, et al. Clinical significance of extrapancreatic lesions in autoimmune pancreatitis. Pancreas. 2010;39(1):e1–5.
Cheuk W, Yuen HKL, Chu SYY, Chiu EKW, Lam LK, Chan JKC. Lymphadenopathy of IgG4-related sclerosing disease. Am J Surg Pathol. 2008;32(5):671–81.
Hamano H, Arakura N, Muraki T, Ozaki Y, Kiyosawa K, Kawa S. Prevalence and distribution of extrapancreatic lesions complicating autoimmune pancreatitis. J Gastroenterol. 2006;41(12):1197–205.
Nakajo M, Jinnouchi S, Fukukura Y, Tanabe H, Tateno R, Nakajo M. The efficacy of whole-body FDG-PET or PET/CT for autoimmune pancreatitis and associated extrapancreatic autoimmune lesions. Eur J Nucl Med Mol Imaging. 2007;34(12):2088–95.
Hamed G, Tsushima K, Yasuo M, et al. Inflammatory lesions of the lung, submandibular gland, bile duct and prostate in a patient with IgG4-associated multifocal systemic fibrosclerosis. Respirology. 2007;12(3):455–7.
Smyrk TC. Pathological features of IgG4-related sclerosing disease. Curr Opin Rheumatol. 2011;23(1):74–9.
Takato H, Yasui M, Ichikawa Y, et al. Nonspecific interstitial pneumonia with abundant IgG4-positive cells infiltration, which was thought as pulmonary involvement of IgG4-related autoimmune disease. Intern Med. 2008;47(4):291–4.
Saegusa H, Momose M, Kawa S, et al. Hilar and pancreatic gallium-67 accumulation is characteristic feature of autoimmune pancreatitis. Pancreas. 2003;27(1):20–5.
Yokoyama A, Kondo K, Nakajima M, et al. Prognostic value of circulating KL-6 in idiopathic pulmonary fibrosis. Respirology. 2006;11(2):164–8.
Al-Salmi QA, Walter JN, Colasurdo GN, et al. Serum KL-6 and surfactant proteins A and D in pediatric interstitial lung disease. Chest. 2005;127(1):403–7.
Kinoshita F, Hamano H, Harada H, et al. Role of KL-6 in evaluating the disease severity of rheumatoid lung disease: comparison with HRCT. Respir Med. 2004;98(11):1131–7.
Kamisawa T, Shimosegawa T, Okazaki K, et al. Standard steroid treatment for autoimmune pancreatitis. Gut. 2009;58(11):1504–7.
Khan ML, Colby TV, Viggiano RW, Fonseca R. Treatment with bortezomib of a patient having hyper IgG4 disease. Clin Lymphoma Myeloma Leuk. 2010;10(3):217–19.
Ghazale A, Chari ST, Zhang L, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology. 2008;134(3):706–15.
Khosroshahi A, Bloch DB, Deshpande V, Stone JH. Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4-related systemic disease. Arthritis Rheum. 2010;62(6):1755–62.
Takahashi N, Ghazale AH, Smyrk TC, Mandrekar JN, Chari ST. Possible association between IgG4-associated systemic disease with or without autoimmune pancreatitis and non-Hodgkin lymphoma. Pancreas. 2009;38(5):523–6.
Fukui T, Mitsuyama T, Takaoka M, Uchida K, Matsushita M, Okazaki K. Pancreatic cancer associated with autoimmune pancreatitis in remission. Intern Med. 2008;47(3):151–5.
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Ryu, J.H., Sekiguchi, H., Yi, E.S. (2013). IgG4-Related Lung Disease. In: Levy, M., Chari, S. (eds) Autoimmune (IgG4-related) Pancreatitis and Cholangitis. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-6430-4_19
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DOI: https://doi.org/10.1007/978-1-4419-6430-4_19
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