Abstract
We report a 72-year-old man with respiratory involvement of immunoglobulin G4 (IgG4)-related disease, who developed dry cough and shortness of breath on effort. The chest computed tomography scan image showed massive and diffuse ground-glass opacity, interlobular thickening, and bronchial wall thickening. The infiltration of IgG4-positive plasma cells in the transbronchial lung biopsy and high serum IgG4 concentrations were found. The patient was treated with 0.6 mg/kg oral prednisolone and showed rapid improvement. This is a case of IgG4-related disease in which the only complication was respiratory involvement.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001;344:732–8.
Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, et al., The Research Program for Intractable Disease by Ministry of Health, Labor and Welfare (MHLW) Japan G4 team. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol. 2011.doi:10.1007/s10165-011-0508-6.
Ito M, Yasuo M, Yamamoto H, Tsushima K, Tanabe T, Yokoyama T, et al. Central airway stenosis in a patient with autoimmune pancreatitis. Eur Respir J. 2009;33:680–3.
Takato H, Yasui M, Ichikawa Y, Fujimura M, Nakao S, Zen 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;47:291–4.
Taniguchi T, Ko M, Seko S, Nishida O, Inoue F, Kobayashi H, et al. Interstitial pneumonia associated with autoimmune pancreatitis. Gut. 2004;53:770.
Zen Y, Kitagawa S, Minato H, Kurumaya H, Katayanagi K, Masuda S, et al. IgG4-positive plasma cells in inflammatory pseudotumor (plasma cell granuloma) of the lung. Hum Pathol. 2005;36:710–7.
Rossi G, Marchioni A, Guicciardi N, Cadioli A, Cavazza A. Recurrent pleural and pericardium effusions in a white woman with IgG4-related syndrome. Am J Surg Pathol. 2009;33:802–3.
Ando N, Yasuda I, Saito M, Moriwaki H. Hilar lymphadenopathy associated with autoimmune pancreatitis. Pancreas. 2006;33:101–2.
Hirano K, Kawabe T, Komatsu Y, Matsubara S, Togawa O, Arizumi T, et al. High-rate pulmonary involvement in autoimmune pancreatitis. Intern Med J. 2006;36:58–61.
Masaki Y, Dong L, Kurose N, Kitagawa K, Morikawa Y, Yamamoto M, et al. Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders. Ann Rheum Dis. 2009;68:1310–5.
Matsui S, Taki H, Shinoda K, Suzuki K, Hayashi R, Tobe K, et al. Respiratory involvement in IgG4-related Mikulicz’s disease. Mod Rheumatol. [Epub ahead of print].
Toyoshima M, Chida K, Kono M, et al. IgG4-related lung disease in a worker occupationally exposed to asbestos. Intern Med. 2010;49:1175–8.
Inoue D, Zen Y, Abo H, Gabata T, Demachi H, Kobayashi T, et al. Immunoglobulin G4-related lung disease: CT findings with pathologic correlations. Radiology. 2009;251:260–70.
Tsushima K, Tanabe T, Yamamoto H, Koizumi T, Kawa S, Hamano H, et al. Pulmonary involvement of autoimmune pancreatitis. Eur J Clin Invest. 2009;39:714–22.
Zen Y, Inoue D, Kitao A, Onodera M, Abo H, Miyayama S, et al. IgG4-related lung and pleural disease: a clinicopathologic study of 21 cases. Am J Surg Pathol. 2009;33:1886–93.
Acknowledgments
This work is supported by Intractable Diseases, the Health and Labor Sciences Research Grants from Ministry of Health, Labor and Welfare.
Conflict of interest
None.
Author information
Authors and Affiliations
Corresponding author
About this article
Cite this article
Umeda, M., Fujikawa, K., Origuchi, T. et al. A case of IgG4-related pulmonary disease with rapid improvement. Mod Rheumatol 22, 919–923 (2012). https://doi.org/10.1007/s10165-012-0600-6
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10165-012-0600-6