title: Types of Organ Involvement in Patients with Immunoglobulin G4-related Disease.
abstract: BACKGROUND
  Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized systemic disease that can involve multiple organs and various clinical phenotypes. The purpose of this study was to analyze different types of organ involvement in IgG4-RD patients in China.
  
  
  METHODS
  We conducted a prospective cohort study on IgG4-RD patients to analyze the clinical manifestations and rare features of IgG4-RD. Patients were grouped into different types according to organ involvement regarding organ number and organ site. The constituent ratio in different types was also analyzed.
  
  
  RESULTS
  A total of 200 IgG4-RD patients, with a male:female ratio of 2.08:1, were grouped into different types. Cases having involvement of two or three organs were the most common whereas the fewest number of patients had multi-organ (≥4) involvement. Serum IgG4 and IgE levels, IgG4/IgG ratio, and percentage of eosinophils increased as the number of involved organs increased. In addition, constituent ratio analysis revealed that patients with salivary gland/lacrimal gland swelling, who also constituted the largest number of IgG4-RD patients, had higher serum IgG4 concentrations and IgG4/IgG values, had higher percentage of Eos, and were more likely to have had a history of allergies relative to patients with internal organ involvement.
  
  
  CONCLUSIONS
  The characteristic feature of IgG4-RD is multiple organ involvement with various clinical manifestations and different types. Although serum IgG4 levels increased with the number of involved organs, serum IgG4 levels were higher for those patients with salivary gland/lacrimal gland swelling compared with those with internal organ involvement. Thus, valuable clues to the differential diagnosis of IgG4-RD could be obtained by examining the clinical patterns of organ involvement.
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100032, China.
authors.forename: Yu
authors.initials: Y
authors.lastname: Chen
authors.affiliation: Department of Stomatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
authors.forename: Ji-Zhi
authors.initials: JZ
authors.lastname: Zhao
authors.affiliation: Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
authors.forename: Rui-E
authors.initials: RE
authors.lastname: Feng
authors.affiliation: Department of Respiratory, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
authors.forename: Ju-Hong
authors.initials: JH
authors.lastname: Shi
authors.affiliation: Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
authors.forename: Xue-Mei
authors.initials: XM
authors.lastname: Li
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100032, China.
authors.forename: Yun-Yun
authors.initials: YY
authors.lastname: Fei
authors.affiliation: Department of Rheumatology, Traditional Chinese Medicine Hospital, Capital Medical University, Beijing 100010, China.
authors.forename: Yang
authors.initials: Y
authors.lastname: Shi
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100032, China.
authors.forename: Wen
authors.initials: W
authors.lastname: Zhang
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100032, China.
authors.forename: Feng-Chun
authors.initials: FC
authors.lastname: Zhang
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000150:complications / Q000473:pathology*; D004804:Eosinophils / Q000378:metabolism; D005260:Female; D006801:Humans; D007073:Immunoglobulin E / Q000097:blood; D007074:Immunoglobulin G / Q000097:blood*; D007668:Kidney / Q000473:pathology; D007765:Lacrimal Apparatus / Q000473:pathology; D008168:Lung / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010902:Pituitary Gland / Q000473:pathology; D011446:Prospective Studies; D011467:Prostate / Q000473:pathology; D012469:Salivary Glands / Q000473:pathology; D013961:Thyroid Gland / Q000473:pathology; D014599:Uterus / Q000473:pathology; D055815:Young Adult
chemical_list: D007074:Immunoglobulin G; D007073:Immunoglobulin E