Tuberculosis and Respiratory Diseases

The Korean Academy of Tuberculosis and Respiratory Diseases (대한결핵및호흡기학회)
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Two Cases of Pulmonary Involvement of Immunoglobulin G4 Related Autoimmune Disease

면역글로불린 G4 연관 자가 면역 질환의 폐 침범 2예

  • Yoo, Jung-Wan (Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Roh, Jae-Hyung (Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Lim, Chae-Man (Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Lee, Sang-Do (Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kim, Woo Sung (Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kim, Dong Soon (Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Song, Jin Woo (Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine)
  • 유정완 (울산대학교 의과대학 서울아산병원 호흡기내과학교실) ;
  • 노재형 (울산대학교 의과대학 서울아산병원 호흡기내과학교실) ;
  • 임채만 (울산대학교 의과대학 서울아산병원 호흡기내과학교실) ;
  • 이상도 (울산대학교 의과대학 서울아산병원 호흡기내과학교실) ;
  • 김우성 (울산대학교 의과대학 서울아산병원 호흡기내과학교실) ;
  • 김동순 (울산대학교 의과대학 서울아산병원 호흡기내과학교실) ;
  • 송진우 (울산대학교 의과대학 서울아산병원 호흡기내과학교실)
  • Received : 2009.06.29
  • Accepted : 2009.08.17
  • Published : 2009.10.30
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Abstract

Immunoglobulin G4 (IgG4) related autoimmune diseases are characterized by high serum IgG4 concentrations, sclerosing inflammation of numerous IgG4-positive lymphoplasma cells of varying origin, and a positive response to steroid treatment. Autoimmune pancreatitis, sclerosing cholangitis, and retroperitoneal fibrosis are representative presentations of IgG4 related autoimmune disease. Herein, we describe 2 patients (40-years-old woman and 47-years-old man) diagnosed with pulmonary involvement of IgG4-related autoimmune disease. The patients were admitted for an evaluation of the lung mass or multiple lung nodules found on chest radiography. Surgical lung biopsies were performed and pathologic finding revealed lymphoplasmacytic sclerosing inflammation with numerous IgG4 positive cells. The patients had elevated serum total IgG and IgG4 levels. Treatment consisted of high dose methylpredinisolone (1 mg/kg/day) and demonstrated good responsiveness. However, one patient experienced 2 relapses while being tapered off of steroid treatment.

Keywords

References

  1. Kamisawa T, Funata N, Hayashi Y, Eishi Y, Koike M, Tsuruta K, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol 2003;38:982-4 https://doi.org/10.1007/s00535-003-1175-y
  2. 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 https://doi.org/10.1016/j.humpath.2005.05.011
  3. Hamed G, Tsushima K, Yasuo M, Kubo K, Yamazaki S, Kawa S, et al. Inflammatory lesions of the lung, submandibular gland, bile duct and prostate in a patient with IgG4-associated multifocal systemic fibrosclerosis. Respirology 2007;12:455-7 https://doi.org/10.1111/j.1440-1843.2007.01053.x
  4. 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 https://doi.org/10.2169/internalmedicine.47.0411
  5. Duvic C, Desrame J, Leveque C, Nedelec G. Retroperitoneal fibrosis, sclerosing pancreatitis and bronchiolitis obliterans with organizing pneumonia. Nephrol Dial Transplant 2004;19:2397-9 https://doi.org/10.1093/ndt/gfh050
  6. 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 https://doi.org/10.1056/NEJM200103083441005
  7. 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 https://doi.org/10.1111/j.1445-5994.2006.01009.x
  8. Taniguchi T, Ko M, Seko S, Nishida O, Inoue F, Kobayashi H, et al. Interstitial pneumonia associated with autoimmune pancreatitis. Gut 2004;53:770; author reply 770-1
  9. 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 https://doi.org/10.1148/radiol.2511080965
  10. Kobayashi H, Shimokawaji T, Kanoh S, Motoyoshi K, Aida S. IgG4-positive pulmonary disease. J Thorac Imaging 2007;22:360-2 https://doi.org/10.1097/RTI.0b013e31813fab9f

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