Abstract
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.
Clinicopathological Characteristics of LgG4-Related Lung Disease