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Journal of Peking University (Health Sciences) ›› 2023, Vol. 55 ›› Issue (6): 1028-1032. doi: 10.19723/j.issn.1671-167X.2023.06.011

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Medical visit status and clinical features in patients with IgG4 related disease

Lu FENG1,2,Jia-yu ZHAI1,Jin-xia ZHAO1,*()   

  1. 1. Department of Rheumatology and Immunology, Peking University Third Hospital, Beijing 100191, China
    2. Department of Rheumatology and Immunology, Liaoning Health Industry Group Bengang General Hospital, Benxi 117000, Liaoning, China
  • Received:2023-08-18 Online:2023-12-18 Published:2023-12-11
  • Contact: Jin-xia ZHAO E-mail:zhao-jinxia@163.com

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Abstract:

Objective: To understand the medical treatment and clinical characteristics of patients with IgG4-related disease (IgG4-RD) with complex clinical manifestations and easy to be misdiagnosed and missed, and to improve the recognition of this disease among doctors from relevant medical departments. Methods: A retrospective analysis was conducted on the medical records of patients diagnosed with IgG4-RD who were hospitalized and discharged from Peking University Third Hospital from January 1, 2012 to December 31, 2022. The patient' s medical visit status, clinical manifestations, laboratory examinations, diagnosis, and treatment information were summarized. Results: A total of 116 patients diagnosed with IgG4-RD were included in this study, with a male to female ratio of 2. 52∶ 1 and an average age of (61.83±10.80) years. The departments for initial visits were gastroenterology, general surgery, and ophthalmology. While the departments responsible for definitive diagnosis were gastroenterology, rheumatology and immunology, and respiratory medicine. Twenty-one patients (18. 10%) required consultation and treatment from three or more departments before receiving a definitive diagnosis. The median time from symptom onset to the initial clinic visit was 2 (1, 7) months, and the median time from symptom onset to diagnosis was 1 (1, 12) month. Twenty-four patients (20.69%) underwent surgical resection of the affected sites before diagnosis. According to the classification criteria of IgG4-RD, sixty-eight (58.62%) cases were diagnosed definitively, eight (6.9%) cases were likely to be diagnosed, and 40 (34.48%) cases were suspected to be diagnosed. In the 68 definitively diagnosed patients, the most commonly affected organs were submandibular gland, the pancreas, biliary tract, parotid in sequence. The median serum IgG4 (IgG4, immunoglobulin G4) level was 6.16 (3. 61, 12. 30) g/L. Fifty-seven patients (83.82%) were treated with glucocorticoids, and 14 patients (20.59%) were treated with immunosuppressants. The use of immunosuppressants was mainly in the rheumatology and immunology department (78. 57%). Conclusion: IgG4-RD is more common in elderly males, with submandibular gland, the pancreas, biliary tract, and parotid being most commonly affected. The distribution of initial visit departments in patients is wide. The proportion of definitive diagnosis based on pathology is relatively low. In terms of treatment, the main approach is steroid treatment, while the use of immunosuppres-sants is not widespread.

Key words: IgG4-related disease, Medical visit status, Clinical characteristics

CLC Number: 

  • R593.2

Figure 1

The initial visit and confirmed departments of 116 patients"

Table 1

Laboratory examinations of 68 patients with definitively diagnosed IgG4-RD"

Items n Value Percent
IgG4/(g/L), M(P25, P75) 68 6.16 (3.61, 12.30) 95.50*
IgG/(g/L), M(P25, P75) 61 19.00(15.55, 20.05) 70.49*
IgA/(g/L), M(P25, P75) 60 1.96 (1.43, 2.71) 1.70*
IgM/(g/L), M(P25, P75) 60 0.88 (0.60, 1.31) 1.70*
IgE/(IU/mL), M(P25, P75) 58 457.90(201.08, 1 110.00) 89.66*
C3/(g/L), ${\bar x}$±s 59 0.85±0.27 59.32#
C4/(g/L), M(P25, P75) 59 0.16 (0.12, 0.22) 25.42#
ESR/(mm/h), M(P25, P75) 57 26.50 (14.00, 48.75) 68.42*
CRP/(g/L), M(P25, P75) 56 0.38 (0.09, 42.00) 28.57*
RF/(IU/mL), M(P25, P75) 26 20.00 (20.00, 38.50) 38.43*

Table 2

Comparison of clinical features between patients with definitive diagnosed and likely/suspected diagnosed IgG4-RD"

Items Definitive diagnosed Likely and suspected diagnosed Z/t/χ2 P value
Male∶Female 2.09∶1 3.36∶1 1.231 0.267
Average age/years, ${\bar x}$±s 61.60±9.62 62.79±12.31 2.963 0.978
Submandibular gland affected/% 57.35 39.58 2.399 0.121
Pancreas affected/% 48.53 70.83 5.737 0.017*
Biliary tract affected/% 30.88 33.33 0.006 0.938
Parotid affected/% 30.88 31.25 0.002 0.966
Lymph gland affected/% 27.94 35.42 4.548 0.033*
Lacrimal affected/% 27.94 12.50 3.968 0.046*
IgG4/(g/L), M(P25，P75) 6.16 (3.61, 12.30) 4.03 (3.56, 15.20) -0.106 0.916
IgG/(g/L), M((P25，P75) 19.00 (15.55, 20.05) 19.55 (16.30, 24.13) -0.681 0.496
IgA/(g/L), M((P25，P75) 1.96 (1.43, 2.71) 1.90 (1.19, 2.63) -0.446 0.656
IgM/(g/L), M((P25，P75) 0.88 (0.60, 1.31) 0.75 (0.53, 0.92) -1.368 0.171
IgE/(IU/mL), M((P25，P75) 457.90(201.08, 1 110.00) 642.45 (192.90, 1 190.00) -0.521 0.603
C3/(g/L), ${\bar x}$±s 0.85±0.27 0.83±0.31 0.204 0.834
C4/(g/L), M((P25，P75) 0.16 (0.12, 0.22) 0.18 (0.13, 0.26) -1.305 0.192
ESR/(mm/h), M((P25，P75) 26.50 (14.00, 48.75) 35.00 (18.25, 58.25) -1.393 0.614
CRP/(g/L), M((P25，P75) 0.38 (0.09, 42.00) 0.42 (0.24, 0.91) -0.163 0.871
RF/(IU/mL), M((P25，P75) 20.00 (20.00, 38.50) 20.00 (20.00, 26.80) -0.497 0.619
Glucocorticoids trerapy/% 83.82 75.00 1.378 0.240
Immunosuppressants trerapy/% 20.59 18.75 0.060 0.807
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