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Research Article
Lingyun Wang
Sun Yat-sen Memorial Hospital, Sun Yat-sen University
Corresponding Author
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Background: Immunoglobulin G4-related lung disease (IgG4-RLD) is a rare entity. The aim of this study is to retrospectively analyzed the clinicopathological characteristics of IgG4-RLD pathologically confirmed in order to improve the diagnosis and treatment of the disease.
Methods: We screened the pathological reports of 4838 patients with pulmonary surgery and/or biopsy specimens at Sun Yat-sen Memorial Hospital affiliated to Sun Yat-sen University from April 2017 to April 2021, and 65 patients with suspected IgG4-RLD had an immunohistochemical staining for IgG4 and IgG. Finally, 10 patients with definite IgG4-RLD pathologically comfirmed were enrolled and analyzed.
Results: The incidence of IgG4-RLD pathologically confirmed was 0.2% (10/4838). Ten patients had a mean age of 59.7 years at diagnosis with a male: female ratio of 9:1. The initial clinical manifestations were lack of specificity and cough was the most common symptom (4/10). More than one organ was involved in most patients (8/10) and lymph node involvement was often seen (7/10). Serum IgG4 were detected and elevated in 6 patients. The levels of serum tumor markers were within normal range or slightly elevated. The chest computed tomography (CT) and/or 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET-CT) findings showed 5 patients of mix type, 3 patients of solid nodular type and 2 patients of bronchovascular type. All pulmonary mass and large nodules with solid pattern had spiculated margins and inhomogeneous enhancement with or without pleural indentation and lobulated appearance. Abundant lymphoplasmacytic infiltration and fibrosis were observed in all patients. The expressions of IgG4 and IgG in the plasma cells were up-regulated in the immunostained sections of lung. Seven patients received prednisone with or without additional immunosuppressive drugs and responded well.
Conclusions: Our study suggests that multiple imaging findings combined with elevated serum IgG4 as well as serum tumor markers without significant increase could provide diagnostic support for IgG4-RLD.
Keywords
Immunoglobulin G4-related disease, Immunoglobulin G4-related lung disease, Clinical characteristics, Pathology, Chest computed tomography
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No competing interests reported.
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This preprint is under consideration at BMC Pulmonary Medicine. A preprint is a preliminary version of a manuscript that has not completed peer review at a journal. Research Square does not conduct peer review prior to posting preprints. The posting of a preprint on this server should not be interpreted as an endorsement of its validity or suitability for dissemination as established information or for guiding clinical practice.
Learn more about In Review
Research Article
Lingyun Wang
Sun Yat-sen Memorial Hospital, Sun Yat-sen University
Corresponding Author
Badges
Prescreen
This manuscript passed our Prescreen assessment
Complete author information
Appropriate declaration statements
Potential risks to human health
Prescreen
Peer Review Timeline
CURRENT STATUS: Under Review
Version 1
Posted 08 Nov, 2021
No community comments so far
Editorial decision: Major revision
On 02 Nov, 2021
Reviews received
Received 30 Oct, 2021
Reviewers agreed
On 30 Oct, 2021
Reviewers agreed
On 27 Oct, 2021
Reviewers invited
Invitations sent on 27 Oct, 2021
Editor assigned
On 27 Oct, 2021
Editor invited
On 27 Oct, 2021
Submission checks complete
On 27 Oct, 2021
First submitted
On 26 Oct, 2021
Metrics
Comments: 0
PDF Downloads: ...
HTML Views: ...
Subject Areas
Pulmonology
License:
This work is licensed under a CC BY 4.0 License. Read Full License
Background: Immunoglobulin G4-related lung disease (IgG4-RLD) is a rare entity. The aim of this study is to retrospectively analyzed the clinicopathological characteristics of IgG4-RLD pathologically confirmed in order to improve the diagnosis and treatment of the disease.
Methods: We screened the pathological reports of 4838 patients with pulmonary surgery and/or biopsy specimens at Sun Yat-sen Memorial Hospital affiliated to Sun Yat-sen University from April 2017 to April 2021, and 65 patients with suspected IgG4-RLD had an immunohistochemical staining for IgG4 and IgG. Finally, 10 patients with definite IgG4-RLD pathologically comfirmed were enrolled and analyzed.
Results: The incidence of IgG4-RLD pathologically confirmed was 0.2% (10/4838). Ten patients had a mean age of 59.7 years at diagnosis with a male: female ratio of 9:1. The initial clinical manifestations were lack of specificity and cough was the most common symptom (4/10). More than one organ was involved in most patients (8/10) and lymph node involvement was often seen (7/10). Serum IgG4 were detected and elevated in 6 patients. The levels of serum tumor markers were within normal range or slightly elevated. The chest computed tomography (CT) and/or 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET-CT) findings showed 5 patients of mix type, 3 patients of solid nodular type and 2 patients of bronchovascular type. All pulmonary mass and large nodules with solid pattern had spiculated margins and inhomogeneous enhancement with or without pleural indentation and lobulated appearance. Abundant lymphoplasmacytic infiltration and fibrosis were observed in all patients. The expressions of IgG4 and IgG in the plasma cells were up-regulated in the immunostained sections of lung. Seven patients received prednisone with or without additional immunosuppressive drugs and responded well.
Conclusions: Our study suggests that multiple imaging findings combined with elevated serum IgG4 as well as serum tumor markers without significant increase could provide diagnostic support for IgG4-RLD.
Figure 1
Figure 2
Figure 3
No competing interests reported.
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