Chest CT Findings for IgG4-related Disease

doi:10.21203/rs.3.rs-3812318/v1

Objective To investigate the multi-slice spiral CT (MSCT) findings of chest involvement in IgG4-related diseases, and to improve doctors' understanding of this disease.

Methods A retrospective analysis was carried out on the clinical and imaging data of 67 patients with clinically confirmed or suspected IgG4-related diseases.

Results: A total of 60 patients (89.60%) had abnormal chest CT. Among them, 47 patients (70.10%) had enlarged mediastinal lymph nodes. Thickening of the tracheal and tracheobronchial perivascular wall was found in 35 cases (52.20%). Nodules were found in 29 cases (43.30%). There was patch or ground-glass density in 15 cases (22.40%), bilateral enlarged axillary lymph nodes in 9 cases (13.40%), bilateral enlarged hilar lymph nodes in 3 cases (4.50%) and interstitial changes in 8 cases (11.90%). Pleural effusion occurred in 5 cases (7.46%, 2 cases of bilateral and 3 cases of unilateral), and pericardial effusion in 3 cases (4.50%). Seven cases (10.45%) showed no obvious abnormality. The abnormally elevated IgG4 (>135mg/dL) was positively correlated with the thickening of the tracheal and tracheobronchial wall (r = 0.328, p = 0.007) and the enlargement of mediastinal lymph nodes (r = -0.252, p = 0.039); Logistic regression model 1 showed that the incidence of lung as the first symptom was increased in patients with bilateral enlarged hilar lymph node on chest images (OR = 16. 000, 95% CI: 1.280-200.010).

Conclusion: The abnormal manifestations of chest lesions, especially lung involvement, on IgG4-RD were varied. Peribronchovascular involvement and lymph node enlargement are the most common manifestations. Chest CT examination is of great significance in the diagnosis and follow-up of IgG4-RLD.

Biological sciences/Immunology

Health sciences/Diseases

Health sciences/Medical research

Health sciences/Signs and symptoms

IgG4 related diseases

IgG4 related lung diseases

multi-slice spiral CT

multi-slice spiral CT imaging manifestations

QUESTION:What are the imaging findings of igg4-RLD? And is it related to other systems of IgG4 -RD?

PERTINENT FINDINGS: A retrospective analysis was carried out on the clinical and imaging data of 67 patients with clinically confirmed or suspected IgG4-related diseases. A total of 60 patients (89.60%) had abnormal chest CT. Peribronchovascular involvement and lymph node enlargement are the most common manifestations. Chest CT scan should be performed in time when there appear ocular symptoms, bilateral enlarged hilar lymph node, pancreatitis, pituitary adenitis, Gaokang arteritis or abnormally elevated IgG4 (> 135mg/dL), while IgG4-RLD should be considered.

IMPLICATIONS FOR PATIENT CARE:Chest CT examination is of great significance in the diagnosis and follow-up of IgG4-RLD.

IgG4-related disease (IgG4-RD) is an immune-mediated disease that can cause fibrous inflammation of almost any organ (1) and the etiology remains unknown till present. IgG4-RD is characterized by elevated serum IgG4 and extensive infiltration of plasma cells with positive IgG4. IgG4-RD involves multiple organs, including the lacrimal gland, salivary gland, lungs, pancreas, gallbladder, kidney and retroperitoneal fibrosis (RPF). Among these different lesions, up to 35% of IgG4-RD patients (2) have abnormal chest imaging examination, including pulmonary nodules, thickened bronchial vascular bundle, thickened bronchial wall, mass, consolidation, thickened pleura, pleural effusion, aortitis, sclerosing pericarditis, lymphadenopathy and paravertebral mass. Other reports found chest involvement in about 14% of patients by chest imaging examination, among which salivary glands and systemic involvement were more often seen (3, 4). Zen et al. (5, 6) confirmed that IgG4-RD can occur in lung tissue in 2005 and named it IgG4-related lung disease (IgG4-RLD) in 2009. At the International Symposium on IgG4-RD held in Boston in October 2011, IgG4-RD involving lungs was recommended to be called IgG4-RLD (7). This article reviewed the chest imaging manifestations of IgG4-RD related diseases, combined with clinical laboratory examination, to improve the understanding of imaging and clinical doctors to this disease, and thus they could formulate more reasonable treatment plans and follow-up work.

From January 2012 to December 2021, 67 patients with IgG4-RD who were diagnosed or suspected in various departments of the First Medical Center of PLA General Hospital and underwent chest CT examinations were continuously collected. This research was approved by the Medical Ethics Committee of the First Medical Center of PLA General Hospital and conforms to the principles outlined in the Declaration of Helsinki.All patients gave informed consent.

1.1 Clinical data Consecutive 67 patients with IgG4-RD who were diagnosed or suspected and underwent chest CT examination were collected from various departments of the First Medical Center of PLA General Hospital from January 2012 to December 2021, including 13 cases of plain-enhanced chest CT scan and 54 cases of plain chest CT scan. This article analyzed the patients’ clinical, serological and imaging characteristics and treatment responses (as shown in Figure 1). Clinical data showed that there were 45 males and 22 females in this study, with an M/F ratio of 23:11; the age was 25-80 years old, and the average age was 59.63±10.7 years old. The clinical manifestations were diverse, and almost half of the patients were initially diagnosed as IgG4-RD in the rheumatology department. There might appear clinical symptoms in the chest or not, and the symptoms included cough, shortness of breath, chest pain and asthma The average duration of symptoms before diagnosis was 20 months (ranging from 1 to 204 months). Nearly half of the patients (25/67) had a history of smoking. Exclusion criteria: (1) Exclude patients with other lung diseases: including lung cancer, lung infection and interstitial lung disease. (2) Exclude other diseases (such as Sjogren's syndrome, etc.) that can cause lung involvement similar to IgG4-RD. (3) Exclude patients with missing chest CT scans or unable to be re-examined.

Chest findings of IgG4-RD in this study included mediastinal lymphadenopathy, fibrotic mediastinitis, pleural lesions, airway or pulmonary parenchymatous diseases, and pericardial lesions, which were confirmed by clinical manifestations, serological examination, and imaging or pathological findings.

Fig 1 Inclusion flow of IgG4-RLD patients

2 Diagnosis of IgG4-RD

IgG4-RD is an autoimmune disease with multiple organ involvement and different clinical manifestations. Its diagnosis is a comprehensive result based on clinical, serological, radiological and histopathological findings. None of these findings alone provide clear evidence for diagnosis. Cross-sectional imaging (ultrasound, CT and MRI) is an important part of the diagnosis and treatment of IgG4-RD. In this study, patients were classified according to the comprehensive diagnostic criteria of IgG4-RD in 2011 and the classification criteria of American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) in 2019 (score 20) [8, 9]. Age, gender, time of diagnosis, organ involvement, biological assessment, treatment, and outcomes were collected.

Chest CT examination of confirmed and suspected patients was performed according todiagnostic criteria of IgG4-RLD reported by Matsui et al. (10, 11) (as shown in Table 1). Abnormalities were described according to Fleischner vocabulary (12) as follows: bronchiolitis ( tree-in-bud pattern or alveolar nodules), bronchiectasis, solid nodules, ground-glass opacity (GGO), consolidation, pleural effusion or pleural thickening, peribronchovascular thickening, mediastinal lymph nodes, bronchial mucoid impaction, interlobular septal thickening, honeycomb and grid-like changes, structural deformation and mediastinal fibrosis (paravertebral soft tissue zone).

Table 1. Summary of diagnostic criteria for IgG4-RRD.

1. Abnormal shadow on chest CT

Hilar/mediastinal lymphadenopathy

Thickening of bronchial wall, bronchovascular bundle, interlobular septal wall

Nodular shadow, infiltrative shadow, pleural thickening/effusion

2. Elevated serum IgG4 concentration (≥135 mg/dl)

3. Pathological findings satisfying the following two items or more: (a: ≥3 items, b: 2 items)

1) Dense lymphoplasmacytic cell infiltration into respiratory organ tissues

2) IgG4+IgG+cell ratio >40% and/or >10 IgG4+cells/high power field

3) Obliterative phlebitis or obliterative arteritis

4) Characteristic fibrosis, typically storiform pattern

4. Presence of lesions in the extrathoracic organs satisfying the diagnostic criteria of IgG4-related disease

(reference finding)hypocomplementemia

Definite diagnosis (definite):1+2+3a, 1+2+3b+4

Probable diagnosis (probable):1+2+4,1+2+3b+reference finding.

Possible diagnosis (possible):1+2+3b

1.3 Inspection Methods

All 67 patients underwent plain chest scan or plain chest scan plus enhanced chest scan using Philips Brilliance 256 iCT (Netherlands) or Siemens Sensation Cardiac (Germany). The patient laid in the supine position, with arms raised. The scan was performed in a head-advanced way, ranging from the apex of the lung to 3cm below the diaphragm. The whole chest CT scan was completed when the patient held his breath at the end of inhalation. Scanning parameters: tube voltage 120 kVp, automatic tube current modulation, reconstructive thickness 1.0-1.5 mm. Lung window: the window width was 1600HU and the window position was -600HU; Mediastinal window: the window width was 400HU and the window position was 40HU. Contrast-enhanced scanning was performed with 70-90ml of non-ionic contrast medium (iohexol or iopromide, 300mgI/mL) at a flow rate of 3.5 ml/s. Arterial phase and venous phase scans were performed 25-30s and 60-65s after injection of contrast medium respectively. Conditions of the reexamination were consistent with those of the first scanning. After analysis, the images were sent to the picture archiving and communication system (PACS). Images remained in the original size and were shown randomly. The location, size, and shape of the lesions, lung interface, edge, length of burr, relationship with bronchus, blood vessel and pleura, lesion composition, calcification, surrounding status, pleural effusion, hilar and mediastinal lymph node enlargement were evaluated by two experienced senior radiologists using a double-blind method. When opinions differed, a consensus was reached through consultation.

1.4 Statistics

In this study, SPSS (V24.0) and R (4.0.2) were used for statistical analysis. Quantitative data in normal distribution was described by mean ± standard deviation (SD), quantitative data in non-normal distribution was described by median and quartile spacing (IQRs), and qualitative data was described by %; Spearman correlation analysis (variables that did not follow a normal distribution) was used for correlation analysis between pulmonary imaging abnormalities and initial symptoms and sero-immunity abnormalities. Logistic regression was used to explore the abnormal immune indexes and lung imaging changes that affected the first symptoms of the lung. P < 0.05 referred statistically significant difference.

2.1. Basic information of patients

In this study, 67 patients diagnosed with IgG4-RD were included, including 45 males (67.2%) with an average age of 59.63±10.7 years. Among all the cases, 25 patients were smokers, accounting for 37.3%. The first affected organs was pancreas in 28 patients, accounting for 41.8% of the total number, followed by eyes, bile ducts and lungs (9 cases, 13.4% for each organ), retroperitoneum (8 cases, 11.9%), lymph nodes (5 cases, 7.5%), blood vessels (4 cases, 6.00%), and meninges, connective tissue, nasopharynx, pituitary and kidney (2 cases, 3.00%, for each organ).

The results of serum immune indexes showed abnormally elevated IgE (>100IU/L) in 26 cases, accounting for 39.40% of the total number; abnormally elevated IgG (> 1660g/L) in 39 cases, accounting for 58.20%; abnormally elevated IgG4 (> 135mg/dL) in 62 patients, accounting for 92.50%; and abnormally elevated Crp (> 5mg/L) in 8 cases, accounting for 11.90% of the total population.

Chest imaging results showed that 60 patients (89.60%) had abnormal lungs. Among them, 47 patients (70.10%) had enlarged mediastinal lymph nodes. Thickening of tracheal and tracheobronchial perivascular wall was found in 35 cases (52.20%), including the tree-in-bud sign in 13 cases. There was bronchiectasis in 9 cases, thickening of tracheobronchial wall in 26 cases, consolidation or mucoid impaction in 1 case and nodules in 29 cases (43.30%). The nodules were solid, about 5-15mm in size, with smooth edges and no obvious burrs. There was patch or ground-glass density in 15 cases (22.40%), bilateral enlarged axillary lymph nodes in 9 cases (13.40%), bilateral enlarged hilar lymph nodes in 3 cases (4.50%) and interstitial changes in 8 cases (11.90%). (See Table 2 and Figure 2). Bilateral pleural effusion occurred in 2 cases (3.00%), unilateral pleural effusion in 3 cases (4.50%), and pericardial effusion in 3 cases (4.50%). Seven cases (10.45%) showed no obvious abnormality. According to the classification of chest abnormality, peribronchovascular involvement (52.20%) and lymph node enlargement (70.10%) were the most common, followed by nodules (43.30%) and patches or GGO (22.40%), and interstitial changes (11.90%) were less common. Fifty-three patients (79.10%) had at least two or more chest abnormalities (Table 3).

Table 2 Description of basic data characteristics

	Mean±SD/N(%)
Gender(M, %)	45, 67.20%
Age X±SD	59.63±10.7
Smoking (Smokers, %)	25, 37.30%
First symptom
Eyes, %	9, 13.40%
Blood vessels, %	4, 6.00%
Pancreas, %	28, 41.80%
Meninges, %	2, 3.00%
Bile duct, %	9, 13.40%
Connective tissue, %	2, 3.00%
Lungs, %	9, 13.40%
Retroperitoneal region, %	8, 11.90%
Nasopharynx, %	2, 3.00%
Hypophysis, %	2, 3.00%
Lymph gland, %	5, 7.50%
Kidneys, %	2, 3.00%
Biochemistry index
Abnormal IgE, %	26, 39.40%
Abnormal IgG, %	39, 58.20%
Abnormal IgG4, %	62, 92.50%
Abnormal Crp, %	8, 11.90%
Imaging indexes of lungs
Lung imaging (Abnormal, %)	60, 89.60%
Nodules, %	29, 43.30%
Patches or GGO, %	15, 22.40%
Trachea and bronchus wall thickening, %	35, 52.20%
Interstitial changes, %	8, 11.90%
Enlarged mediastinal lymph nodes, %	47, 70.10%
Enlarged bilateral hilar nodes, %	3, 4.50%
Enlarged bilateral axillary lymph nodes, %	9, 13.40%
Bilateral pleural effusion, %	2, 3.00%
Right pleural effusion, %	3, 4.50%
Pericardial effusion, %	3, 4.50%

Note: Abnormal IgE: IgE > 100IU/L; Abnormal IgG: IgG > 1660g/L; Abnormal IgG4: IgG4 > 135mg/dL; Abnormal Crp: Crp > 5mg/L

Fig. 2 Various imaging patterns of chest patients with IgG4-related diseases (each computed tomography (CT) scan section comes from a different patient). Axial CT scan of lung window a)-e): a) showing multiple nodules of different sizes in both lungs. b) Showing thickening of bronchioles wall in both lungs. c) Showing the consolidation of both lungs along the bronchus. d) Showing a right paraphilar mass. e) Showing small grid density and ground glass density around the lungs, considering interstitial changes. Mediastinal window axial enhanced CT f)-h): showing multiple enlarged lymph nodes in the mediastinum, bilateral hila and bilateral axillary region.

2.2 Correlation between chest imaging and first-involved organ and symptoms

Correlation analysis showed that ocular symptoms were positively correlated with normal lung imaging (r = -0.295, ρ = 0.015); Lung symptoms were positively correlated with bilateral enlarged hilar lymph node(r = 0.338, ρ = 0.005); There was a positive correlation between lymph node symptom and bilateral axillary lymph node enlargement (r = 0.388, ρ = 0.001); Pituitary symptoms (r = 0.386, p = 0.001) and vascular symptoms (r = 0.250, p = 0.041) were positively correlated with right pleural effusion; There was a positive correlation between pancreatic symptoms and pericardial effusion (r = 0.256, p = 0.037), as shown in Figure 3.

Fig. 3 Correlation between lung imaging and first symptom

2.3 Correlation between lung imaging and abnormal serum immune indexes

Correlation analysis showed that abnormally elevated IgG (> 1660g/L) was negatively correlated with right pleural effusion (r = -0.256, ρ = 0.037); abnormally elevated IgG4 (> 135mg/dL) was positively correlated with the thickened tracheobronchial wall (r = 0.328, p = 0.007) and the enlarged mediastinal lymph nodes (r = -0.252, p = 0.039). The results are shown in Figure 4.

Fig. 4 Correlation between lung imaging and abnormal serum immune indexes

2.4 Main indicators affecting the first chest symptoms

Gender, age, smoking status, serum immune related indexes and chest imaging were included in the logistic regression model, and the variables in the model were screened by the forward stepwise regression method. The results showed that only the bilateral enlarged hilar lymph node was included in model 1. Logistic regression model 1 showed that in patients with bilateral enlarged hilar lymph node on chest images, the probability of lung as the first symptom was increased (OR = 16.000, 95% CI: 1.280-200.010); After adjusting gender, age and smoking factors, Logistic regression model 2 showed the same trend (OR = 19.694, 95% CI: 1.148-337.726), and the results were shown in Table 2.

Table 3 Logistic regression analysis of lung as the first symptom

	Model 1		Model 2
	OR value (95%)	P value	OR value (95%)	P value
N	1		1
Y	16.000(1.280-200.010)	0.031	19.694(1.148-337.726)	0.04

Note: Model 1 included bilateral hilar lymph node enlargement; Model 2 included gender, age and smoking based on Model 1

There were 60 cases (89.55%) with abnormal chest images, showing one or more kinds of abnormal chest images, which was consistent with the literature reports. About 17.6% - 40.0% of IgG4-RD patients had lung involvement, while about 37.5% - 87.5% of IgG4-RLD patients had extrapulmonary or extrathoracic lesions (9, 13-17). IgG4-RLD usually coexisted with other systemic diseases outside the chest or lungs, but a few of them can also involve the lungs alone. Therefore, patients with IgG4-RLD whose lung lesions are the first or lung is the single lesion site should be followed up regularly, and other organs outside the lungs may be involved in the later stage of the disease. The clinical and imaging manifestations of IgG4-RLD were nonspecific, and the symptoms depended on the lesion site, including cough, chest pain, dyspnea and hemoptysis. In addition, there may be low fever and weight loss, and about half of the patients had no respiratory symptoms (13-17). In this study, 9 (13.43%) patients were admitted due to lung symptoms, and the main symptoms were cough, expectoration, chest pain, asthma and dyspnea; 58 (86.57%) patients were diagnosed with other organs as the first involved organ, and all patients had two or more organs involved at the time of diagnosis. Scholars at home and abroad have found that middle-aged and elderly men are slightly more common, the incidence ratio of males and females is 2.6 : 1, and the average onset age is 58 years old. It is consistent with the study.

The most common lesions in 67 patients were peribronchovascular involvement (52.20%) and lymph node enlargement (70.10%). According to the main abnormal manifestations of the chest, bronchovascular lesions (56%) and lymph node enlargement (31%) were the most common [10], followed by nodules (43.30%) and patches or GGO (22.40%), while interstitial changes (11.90%) were rare. Lv et al. (18) reported that pleural effusion may be unilateral or bilateral, with or without pericardial effusion. There were 3 cases of unilateral pleural effusion, 2 cases of bilateral pleural effusion and 2 cases of pericardial effusion. The incidence of pleural effusion or pleural thickening and mediastinal fibrosis was lower than that of other lesions involved in lung. Fifty-three patients (79.10%) had at least two or more chest abnormalities. In these abnormal manifestations, no priority correlation was found. In addition, there was no significant difference in gender, age, IgG4 level and smoking condition. After statistical analysis, there was a positive correlation between ocular symptoms and normal lung imaging (r = -0.295, ρ = 0.015); Lung symptoms were positively correlated with bilateral enlarged hilar lymph node(r = 0.338, ρ = 0.005); Pitulitis (r = 0.386, p = 0.001) and arteritis (r = 0.250, p = 0.041) were positively correlated with right pleural effusion; There was a positive correlation between pancreatitis and pericardial effusion (r = 0.256, ρ = 0.037). The abnormal increase of IgG4 (> 135mg/dL) was positively correlated with the thickening of tracheobronchial wall (r = 0.328, p = 0.007) and the enlargement of mediastinal lymph nodes (r = -0.252, p = 0.039).

Some abnormal manifestations are associated with specific extrathoracic organ manifestations: GGO and pancreatitis, peribronchovascular involvement and nephritis, interstitial diseases and eosinophilia and lymph node enlargement with salivary adenitis. Though this association found in limited cases is finite, it can also reflect the changes observed in the pathological changes between organs (19). This relevance is also reflected in this study.

Differential diagnosis of IgG4-RLD includes sarcoidosis (20), ANCA-associated vasculitis, connective tissue disease-associated interstitial pneumonia (21), idiopathic interstitial pneumonia (IIP), lymphoma (22, 23), primary or metastatic lung cancer, Erdheim-Chester disease (24), infection (including bacterial infection and non-mycobacterial or fungal infection), bronchial asthma, Castleman's disease and myofibroblastic tumor (25). To exclude these diagnoses, new biopsies, a review of pathological tissue, or additional laboratory tests may be helpful.

From this study, we believe that chest CT scanning is very useful when IgG4-RD is suspected. We suggest that thoracic CT scanning at the initial stage of the disease should be carefully observed at least to identify peribronchovascular thickening and soft tissue-like band lesions, which can be considered to be more specific and helpful for diagnosis. IgG4-RD with multiple organ involvement is a risk factor for recurrence, and thus timely and effective identification of chest involvement helps judge prognosis.

There are some limitations in this study. First of all, it is a single-center study, and thus there is bias in case collection. Whether the results can be extended to other patients with different backgrounds remains to be determined. Secondly, the number of patients undergoing lung biopsy is relatively small, and tissues for pathological diagnosis are extrathoracic tissue in most cases, which lacks pathological records related to the lung, and only meets the criteria such as specific swelling/mass and elevated serum IgG4. Therefore, the diagnosis of IgG4-RLD may be overestimated according to symptoms and imaging findings, especially in some patients who have not improved after treatment. Third, due to the lack of lung function tests, it is impossible to evaluate the impact of diseases on lung function. Finally, because the inclusion criteria require an ACR/EULAR score > 20, patients with solitary lung involvement may be omitted.

Chest lesions of IgG4-RD, especially in patients with lung involvement, can show various abnormalities and are likely to be ignored. Peribronchovascular involvement and lymph node enlargement are the most common manifestations. Nodules, interstitial changes, ground-glass density, pleural disease and mediastinal fibrosis are rare, and these symptoms are nonspecific. Chest CT scan should be performed in time when there appear ocular symptoms, bilateral enlarged hilar lymph node, pancreatitis, pituitary adenitis, Gaokang arteritis or abnormally elevated IgG4 (> 135mg/dL), while IgG4-RLD should be considered.

Data availability

Upon a reasonable request, the corresponding author can provide the data supporting the findings of this study.

Acknowledgments

We would like to express special thanks to the doctors at the radiology department of PLA.

Funding

The authors declare that no funds, grants, or other support were received during the preparation of this manuscript.

Author information

Authors and Affiliations

Diagnostic Radiology Department, The First Medical Center of Chinese PLA General Hospital, Beijiing, 100853

Ye Liu , Yongkang Nie

contributions

YeLiucontributed to study design, data collection and interpretation, and drafting and revisions of the manuscript. YongkangNie contributed to drafting and revision of the manuscript. All authors have read and approved the manuscript.

Corresponding author

Correspondence to Yongkang Nie

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

The study was approved by the Ethical Committee of The First Medical Center of The General Hospital of Chinese People's Liberation Army.According to the rules of the hospital’s medical ethics committee.

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No competing interests reported.

Chest CT Findings for IgG4-related Disease

Status:

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Abstract

Figures

Key Points

Introduction

1 Data and Methods

2 Results

2.1. Basic information of patients

3 Discussion

Conclusion

Declarations

References

Additional Declarations

Status:

Version 1