title: [IgG4-related lung disease:a clinical analysis and literature review].
abstract: OBJECTIVE
  To summarize the clinical features, diagnosis and treatment experience of IgG4-related lung disease for the sake of improving clinical understanding of this disease and reducing the misdiagnosis and mistreatment rates.
  
  
  METHODS
  To analyze the general situation, clinical manifestation, laboratory examination, histopathology, therapy and prognosis of 2 patients with IgG4-related lung disease, who were admitted in the department of respiratory diseases at Changhai Hospital. Publications related to IgG4 lung disease were reviewed.
  
  
  RESULTS
  Both patients were male with elevated serum IgG4 level (2.25 g/L and 10 g/L respectively). In one patient, radiologic finding showed solid nodules in the lung with ileocecal involvement. He responded well to glucocorticoid. The other patient's computed tomography of lung demonstrated bronchovascular type. Glucocorticoid therapy was effective to both patients. A total of 69 cases with IgG4-related lung disease were reported worldwide, among whom 39 cases were admitted with chief complaints of respiratory symptoms. However, there were 41 cases suffering extra-pulmonary involvement. Serum IgG4 levels detected in 48 cases were significantly elevated (307-52 500 mg/L). The radiographic pattern of solid nodule type was the most frequent, accounting for 50.7% (35 cases). A total of 31 (44.9%) patients received glucocorticoid therapy with good response and prognosis.
  
  
  CONCLUSIONS
  IgG4-related lung disease is a rare immunological disease lack of specific symptoms. Serum immunological examination, radiographic characteristics and histopathology should be comprehensively considered for diagnosis. Glucocorticoid is so far the most acceptable therapy with good response rate.
authors.forename: Xinghua
authors.initials: X
authors.lastname: Shan
authors.forename: Xiaomeng
authors.initials: X
authors.lastname: Nie
authors.forename: Xiao
authors.initials: X
authors.lastname: Yong
authors.forename: Ying
authors.initials: Y
authors.lastname: Wang
authors.forename: Liang
authors.initials: L
authors.lastname: Chen
authors.forename: Miaoxia
authors.initials: M
authors.lastname: He
authors.forename: Xiaopeng
authors.initials: X
authors.lastname: Yao
authors.forename: Qiang
authors.initials: Q
authors.lastname: Li
authors.affiliation: Department of Respiratory Medicine, Changhai Hospital, Second Military Medical University, Shanghai 200433, China; Email: huangliur@163.com.
authors.forename: Yi
authors.initials: Y
authors.lastname: Huang
mesh_terms: D001249:Asthma / Q000175:diagnosis; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008168:Lung / Q000276:immunology / Q000473:pathology*; D008171:Lung Diseases / Q000150:complications / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D008297:Male; D011379:Prognosis; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G