Figures
- FIGURE 1
Various imaging patterns of immunoglobulin G4-related disease thoracic involvement (each computed tomography (CT) scan section comes from a different patient). a) Axial contrast-enhanced thoracic CT scan showing an important bronchial thickening of the segmental right superior lobe bronchi (arrows). b) Axial unenhanced thoracic CT scan showing nodular ground–glass opacities in the posterior segment of the right superior lobe (arrows). c) Axial enhanced thoracic CT scan showing lymph nodes enlargement in the mediastinum in situation 5 and 10R (arrows). d) Sagittal reconstruction of thoracic CT scan showing distal bronchial distension and traction bronchiectasis in the interstitial lung pattern (circle). e) Axial unenhanced thoracic CT scan showing a mass in the right superior lobe (arrow). f) Sagittal enhanced aortic CT scan showing a stenosis of descending aorta and aortic wall thickening due to posterior mediastinitis (arrow).
Tables
- TABLE 1
Clinical and biological characteristics of 48 patients with immunoglobulin G4-related disease (IgG4-RD) thoracic involvement
Characteristic Patients (n=48) Demographic data Male 39 (81) Median age at onset (years) 60 (23–83) Median diagnostic delay (months) 6 (1–240) Smoker or former smoker 9 (19) History of atopy 18 (37) Organ involvement of IgG4-RD Median number of organs affected 3 (1–6) Lymph nodes 30 (62.5) Pancreatitis 15 (31.2) Tubulointerstitial nephritis 15 (31.2) Sialadenitis 15 (31.2) Retroperitoneal fibrosis 10 (20.8) Sclerosing cholangitis 10 (20.8) Aortitis 8 (16.6) Dacryoadenitis 6 (12.5) Prostatitis 3 (6.2) Biological characteristics Elevated serum IgG (>1400 mg·dL−1) 43 (89.5) Elevated serum IgG4 (>135 mg·dL−1) 43 (89.5) Elevated serum IgG4 >2xN 32 (66.7) Median serum IgG4 level (mg·dL−1) 430 (23–2040) Hypocomplementaemia 13 (27) Elevated CRP (>5 mg·L−1) 39 (81.2) Positive ANA (>1/160) 9 (18.8) Data are presented as n (%) or median (range). ANA: antinuclear antibody; CRP: C reactive protein.
- TABLE 2
Association between immunoglobulin G4-related disease (IgG4-RD) thoracic involvement patterns.
Nodular Interstitial disease Peribroncho vascular involvement Ground-glass opacities Lymph node enlargement Retro mediastinal fibrosis Pleural disease Nodular 7 Interstitial disease 1 7 Peribronchovascular involvement 2 3 27 Ground-glass opacities 0 1 3 5 Lymph node enlargement 3 3 9 0 15 Retromediastinal fibrosis 0 0 0 0 0 2 Pleural disease 0 3 3 1 2 0 4 - TABLE 3
Classification of immunoglobulin G4-related disease (IgG4-RD) thoracic involvement according to the criteria of Corcoran et al. [6]
Category of thoracic involvement Number of patients (group 1/group2) Thoracic patterns Age at diagnosis, years median±sd Male n/N (%) Elevated serum IgG4 level n/N (%) Smoking history >10 pack years n/N (%) Industrial or environmental exposure to asbestos n/N (%) Definite
Biopsy proved thoracic involvement23/48 15 lymph node enlargement
13 peribronchovascular involvements
Seven nodular
Four interstitial disease
Two GGO59±14 20/23 (87) 20/23 (87) 3/23 (13) 0/23 (0) Highly probable
Typical radiology with no alternative explanation and response to treatment9/48 Seven peribronchovascular involvement
Four lymph node enlargement
Three GGO
One nodular
One interstitial disease49±17 8/9 (89) 8/9 (89) 1/9 (11) 0/9 (0) Probable
Typical radiology with no alternative explanation15/48 Eight peribronchovascular involvements
Three lymph node enlargement
Three nodular65±16 10/15 (66) 14/15 (93) 3/15 (20) 0/15 (0) Possible
Radiology potentially consistent with IgG4-RD but plausible alternative cause identified1/48 One interstitial disease 76 1/1 (100) 1/1 (100) 0/1 (0) 1/1 (100) GGO: ground-glass opacity; sd: standard deviation.
- TABLE 4
Clinico-biological characteristics of patients according to the immunoglobulin G4-related disease (IgG4-RD) thoracic pattern.
Characteristic Nodular n=7 n (%) Interstitial disease n=7 n (%) Peribronchovascular involvement n=25 n (%) Round-shaped GGO n=5 n (%) Lymph node enlargement n=15 n (%) Retromediastinal fibrosis n=2 n (%) Pleural disease n=4 n (%) Male 5 (70) 6 (86) 20 (80) 4 (80) 13 (87) 2 (100) 3 (75) Smoking history 2 (29) 0 (0)* 8 (32) 2 (40) 5 (33) 1 (50) 0 (0) Asthma 1 (14) 2 (29)* 8 (32)* 0 (0) 2 (13) 0 (0) 1 (25) Pancreatitis 2 (29) 2 (29) 8 (32%) 4 (80)* 3 (20) 0 (0) 1 (25) Cholangitis 1 (14) 1 (14) 6 (23%) 1 (20) 3 (20) 0 (0) 0 (0) Nephritis 2 (29) 1 (14) 10 (40)* 1 (20) 3 (20) 0 (0) 0 (0) Orbital tumour 0 (0) 0 (0) 3 (12) 1 (20) 1 (7) 0 (0) 0 (0) Dacryoadenitis 2 (29) 0 (0) 4 (16) 1 (20) 4 (27) 0 (0) 0 (0) Sialadenitis 3 (43) 1 (14) 8 (32) 1 (20) 8 (53)* 0 (0) 0 (0) Retroperitoneal fibrosis 2 (29) 1 (14) 5 (20) 0 (0) 2 (13) 0 (0) 1 (25) Aortitis 1 (14) 2 (29) 5 (20) 0 (0) 4 (27) 1 (50) 1 (25) Prostatitis 0 (0) 0 (0) 3 (12) 1 (20) 0 (0) 0 (0) 0 (0) IgG4 >135 mg·dL−1 6 (86) 7 (100) 23 (92) 4 (80) 15 (100) 1 (50) 4 (100) CRP >5 mg·L−1 5 (71) 5 (71) 21 (84) 4 (80) 13 (87) 2 (100) 4 (100) Hypereosinophilia >1500 cells/mm3 1 (14) 5 (71)* 9 (36) 2 (40) 2 (13) 0 (0) 2 (50) EULAR score mean
(min–max)42.3 (35–70) 41.8 (27–64) 41.6 (25–75) 36.5 (25–54) 41.7 (27–70) 42 (39–45) 45 (27–50) GGO: ground-glass opacity; CRP: C reactive protein; EULAR: European League Against Rheumatism. *: p<0.05.
Supplementary Material
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Supplementary tables and figures ERR-0078-2021_Supplementary_tables_and_figures
