IgG4 Related Lung Disease: Two Cases Report and Literature Review_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

LiangXiaoyan ¹ ,  LiHongxia ² , ZuoZhenhua ¹ ,  CaiShaohua ¹

1. Department of Respiratory Medicine in Special Wards, Chinese PLA General Hospital, Beijing, 100853, China;
2. ;

Corresponding author：

LiHongxia, Email: lhxsls@sina.com; CaiShaohua, Email: csh301@163.com

Keywords：

Autoimmune diseases; IgG4 related lung disease

DOI：

10.7507/1671-6205.2014141

Video：

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Objective To summarize the clinical characteristic of IgG4 related lung disease. Methods The clinical manifestation,laboratory examination,imaging,diagnosis and treatment data of 2 patients with IgG4 related lung disease admitted in the PLA General Hospital from January 2000 to January 2014 were collected and analyzed retrospectively.Related literatures were also reviewed. Results The serum IgG level of IgG4 related lung disease might be normal and the levels of IgG1,2,3 and 4 might increase.Some autoantibodies were positive.IgG4 related lung disease could be easily misdiagnosed as Sjogren's syndrome associated with interstitial pneumonia when the lacrimal gland and salivary gland were involved,and the chest CT occasionally showed multiple vesicles.The immunochemical staining of lung tissues revealed the increase of IgG4/IgG and the number of IgG4 positive lymphatic plasma cells >10/HP.The combination of clinical manifestation,laboratory examination,imaging and pathology results was more conducive for the diagnosis of IgG4 related lung disease.After the treatment of glucocorticoid,the clinical symptoms were relieved and the serum IgG4 level obviously decreased. Conclusion IgG4 related lung disease is rare in clinic,which can involve the lung alone or multiple organs.The increase of IgG4/IgG indicated by the immunochemical staining of lung tissues is the gold standard for diagnosis.Glucocorticoid has good treatment effect in IgG4 related lung disease.

Citation： LiangXiaoyan, LiHongxia, ZuoZhenhua, CaiShaohua. IgG4 Related Lung Disease: Two Cases Report and Literature Review. Chinese Journal of Respiratory and Critical Care Medicine, 2014, 13(6): 579-583. doi: 10.7507/1671-6205.2014141 Copy

1.	Stone JH.IgG4-related disease:nomenclature,clinical features,and treatment.Semi Diagn Pathol,2012,29:177-190.
2.	Duvic C,Desrame J,Leveque C,et al.Retroperitoneal fibrosis,sclerosing pancreatitis and bronchiolitis obliterans with organizing pneumonia.Nephrol Dial Transplant,2004,19:2397-2399.
3.	Umehara H,Okazaki K,Masaki Y,et al.Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD),2011.Rheumatology,2012,22:21-30.
4.	Inoue D,Zen Y,Abe H,et al.Immunoglobulin G4-related lung disease:CT findings with pathologic correlations.Radiology,2009,251:260-270.
5.	Okazaki K,Umehara H.Are classification criteria for igG4-RD now possible? The concept of IgG4-related disease and proposal of comprehensive diagnostic criteria in Japan.Int J Rheumatol,2012,2012:357071.
6.	梅原久范,董凌莉,译.温故而知新:IgG4相关性疾病21世纪被发现的新的疾病体.中华风湿病学杂志,2012,16:848-850.
7.	Yamamoto M,Suzuki C,Naishiro Y,et al.The significance of disease-independence in Mikulicz's disease.Nihon Rinsho Meneki Gakkai Kaishi,2006,29:1-7.
8.	Yamamoto M,Harada S,Ohara M,et al.Clinical and pathological differences between Mikulicz's disease and Sjogren's syndrome.Rheumatology(Oxford),2005,44:227-234.
9.	Ogoshi T,Yatera K,Nagata S,et al.A case of multicentric Castleman disease with massive infiltration of plasmacytes presenting IgG4.Nihon Kokyuki Gakkai Zasshi,2011,49:437-42.
10.	Fujiu K,Sakuma H,Miyamoto H,et al.Immunoglobulin G4-related inflammatory pseudotumor of the lung.Gen Thorac Cardiovasc Surg,2010,58:144-148.
11.	张卉,施举红,冯瑞娥,等.IgG4相关非特异性间质性肺炎四例临床病理分析.中华结核和呼吸杂志,2012,35:747-751.

1. Stone JH.IgG4-related disease:nomenclature,clinical features,and treatment.Semi Diagn Pathol,2012,29:177-190.
2. Duvic C,Desrame J,Leveque C,et al.Retroperitoneal fibrosis,sclerosing pancreatitis and bronchiolitis obliterans with organizing pneumonia.Nephrol Dial Transplant,2004,19:2397-2399.
3. Umehara H,Okazaki K,Masaki Y,et al.Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD),2011.Rheumatology,2012,22:21-30.
4. Inoue D,Zen Y,Abe H,et al.Immunoglobulin G4-related lung disease:CT findings with pathologic correlations.Radiology,2009,251:260-270.
5. Okazaki K,Umehara H.Are classification criteria for igG4-RD now possible? The concept of IgG4-related disease and proposal of comprehensive diagnostic criteria in Japan.Int J Rheumatol,2012,2012:357071.
6. 梅原久范,董凌莉,译.温故而知新:IgG4相关性疾病21世纪被发现的新的疾病体.中华风湿病学杂志,2012,16:848-850.
7. Yamamoto M,Suzuki C,Naishiro Y,et al.The significance of disease-independence in Mikulicz's disease.Nihon Rinsho Meneki Gakkai Kaishi,2006,29:1-7.
8. Yamamoto M,Harada S,Ohara M,et al.Clinical and pathological differences between Mikulicz's disease and Sjogren's syndrome.Rheumatology(Oxford),2005,44:227-234.
9. Ogoshi T,Yatera K,Nagata S,et al.A case of multicentric Castleman disease with massive infiltration of plasmacytes presenting IgG4.Nihon Kokyuki Gakkai Zasshi,2011,49:437-42.
10. Fujiu K,Sakuma H,Miyamoto H,et al.Immunoglobulin G4-related inflammatory pseudotumor of the lung.Gen Thorac Cardiovasc Surg,2010,58:144-148.
11. 张卉,施举红,冯瑞娥,等.IgG4相关非特异性间质性肺炎四例临床病理分析.中华结核和呼吸杂志,2012,35:747-751.

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