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Thoracic involvement and imaging patterns in IgG4-related disease

Romain Muller, Paul Habert, Mikael Ebbo, Julie Graveleau, Mathieu Groh, David Launay, Sylvain Audia, Gregory Pugnet, Fleur Cohen, Antoinette Perlat, Audrey Benyamine, Boris Bienvenu, Lea Gaigne, Pascal Chanez, Jean Yves Gaubert, Nicolas Schleinitz
European Respiratory Review 2021 30: 210078; DOI: 10.1183/16000617.0078-2021
Romain Muller
1Dept of Internal Medicine, Aix Marseille University, APHM, CHU La Timone, Marseille, France
13These authors contributed equally to this work
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  • ORCID record for Romain Muller
Paul Habert
2Dept of Radiology, Aix Marseille University, APHM, CHU La Timone, Marseille, France
13These authors contributed equally to this work
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Mikael Ebbo
1Dept of Internal Medicine, Aix Marseille University, APHM, CHU La Timone, Marseille, France
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Julie Graveleau
3Dept of Internal Medicine, CH Saint Nazaire, Saint Nazaire, France
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Mathieu Groh
4Dept of Internal Medicine, CH Foch, National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France
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David Launay
5Univ. Lille, Inserm, CHU Lille, Service de Médecine Interne et Immunologie Clinique, Centre de référence des maladies autoimmunes systémiques rares du Nord et Nord-Ouest de France (CeRAINO), U1286 - INFINITE - Institute for Translational Research in Inflammation, Lille, France
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Sylvain Audia
6Dept of Internal Medicine, CHU Dijon, Dijon, France
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Gregory Pugnet
7Dept of Internal Medicine and Clinical Immunology, CHU Toulouse, Toulouse, France
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Fleur Cohen
8Dept of Internal Medicine, APHP, CHU Pitié-Salpêtrière, Paris, France
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Antoinette Perlat
9Dept of Internal Medicine, CHU Rennes, Rennes, France
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Audrey Benyamine
10Dept of Internal Medicine, Aix Marseille University, APHM, CHU Nord, Marseille, France
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Boris Bienvenu
11Dept of Internal Medicine, CH St Joseph, Marseille, Marseille, France
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Lea Gaigne
1Dept of Internal Medicine, Aix Marseille University, APHM, CHU La Timone, Marseille, France
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Pascal Chanez
12Dept of Pneumology, Aix Marseille University, APHM, CHU Nord, Marseille, France
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Jean Yves Gaubert
2Dept of Radiology, Aix Marseille University, APHM, CHU La Timone, Marseille, France
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Nicolas Schleinitz
1Dept of Internal Medicine, Aix Marseille University, APHM, CHU La Timone, Marseille, France
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  • For correspondence: nicolas.schleinitz@ap-hm.fr
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    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).

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  • TABLE 1

    Clinical and biological characteristics of 48 patients with immunoglobulin G4-related disease (IgG4-RD) thoracic involvement

    CharacteristicPatients (n=48)
    Demographic data
     Male39 (81)
     Median age at onset (years)60 (23–83)
     Median diagnostic delay (months)6 (1–240)
     Smoker or former smoker9 (19)
     History of atopy18 (37)
    Organ involvement of IgG4-RD
     Median number of organs affected3 (1–6)
     Lymph nodes30 (62.5)
     Pancreatitis15 (31.2)
     Tubulointerstitial nephritis15 (31.2)
     Sialadenitis15 (31.2)
     Retroperitoneal fibrosis10 (20.8)
     Sclerosing cholangitis10 (20.8)
     Aortitis8 (16.6)
     Dacryoadenitis6 (12.5)
     Prostatitis3 (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 >2xN32 (66.7)
     Median serum IgG4 level (mg·dL−1)430 (23–2040)
     Hypocomplementaemia13 (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.

      NodularInterstitial diseasePeribroncho vascular involvementGround-glass opacitiesLymph node enlargementRetro mediastinal fibrosisPleural disease
      Nodular7
      Interstitial disease17
      Peribronchovascular involvement2327
      Ground-glass opacities0135
      Lymph node enlargement339015
      Retromediastinal fibrosis000002
      Pleural disease0331204
    • TABLE 3

      Classification of immunoglobulin G4-related disease (IgG4-RD) thoracic involvement according to the criteria of Corcoran et al. [6]

      Category of thoracic involvementNumber of patients (group 1/group2)Thoracic patternsAge at diagnosis, years median±sdMale 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 involvement
      23/4815 lymph node enlargement
      13 peribronchovascular involvements
      Seven nodular
      Four interstitial disease
      Two GGO
      59±1420/23 (87)20/23 (87)3/23 (13)0/23 (0)
      Highly probable
      Typical radiology with no alternative explanation and response to treatment
      9/48Seven peribronchovascular involvement
      Four lymph node enlargement
      Three GGO
      One nodular
      One interstitial disease
      49±178/9 (89)8/9 (89)1/9 (11)0/9 (0)
      Probable
      Typical radiology with no alternative explanation
      15/48Eight peribronchovascular involvements
      Three lymph node enlargement
      Three nodular
      65±1610/15 (66)14/15 (93)3/15 (20)0/15 (0)
      Possible
      Radiology potentially consistent with IgG4-RD but plausible alternative cause identified
      1/48One interstitial disease761/1 (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.

        CharacteristicNodular 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 (%)
        Male5 (70)6 (86)20 (80)4 (80)13 (87)2 (100)3 (75)
        Smoking history2 (29)0 (0)*8 (32)2 (40)5 (33)1 (50)0 (0)
        Asthma1 (14)2 (29)*8 (32)*0 (0)2 (13)0 (0)1 (25)
        Pancreatitis2 (29)2 (29)8 (32%)4 (80)*3 (20)0 (0)1 (25)
        Cholangitis1 (14)1 (14)6 (23%)1 (20)3 (20)0 (0)0 (0)
        Nephritis2 (29)1 (14)10 (40)*1 (20)3 (20)0 (0)0 (0)
        Orbital tumour0 (0)0 (0)3 (12)1 (20)1 (7)0 (0)0 (0)
        Dacryoadenitis2 (29)0 (0)4 (16)1 (20)4 (27)0 (0)0 (0)
        Sialadenitis3 (43)1 (14)8 (32)1 (20)8 (53)*0 (0)0 (0)
        Retroperitoneal fibrosis2 (29)1 (14)5 (20)0 (0)2 (13)0 (0)1 (25)
        Aortitis1 (14)2 (29)5 (20)0 (0)4 (27)1 (50)1 (25)
        Prostatitis0 (0)0 (0)3 (12)1 (20)0 (0)0 (0)0 (0)
        IgG4 >135 mg·dL−16 (86)7 (100)23 (92)4 (80)15 (100)1 (50)4 (100)
        CRP >5 mg·L−15 (71)5 (71)21 (84)4 (80)13 (87)2 (100)4 (100)
        Hypereosinophilia >1500 cells/mm31 (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.

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        Thoracic involvement and imaging patterns in IgG4-related disease
        Romain Muller, Paul Habert, Mikael Ebbo, Julie Graveleau, Mathieu Groh, David Launay, Sylvain Audia, Gregory Pugnet, Fleur Cohen, Antoinette Perlat, Audrey Benyamine, Boris Bienvenu, Lea Gaigne, Pascal Chanez, Jean Yves Gaubert, Nicolas Schleinitz
        European Respiratory Review Dec 2021, 30 (162) 210078; DOI: 10.1183/16000617.0078-2021

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        Thoracic involvement and imaging patterns in IgG4-related disease
        Romain Muller, Paul Habert, Mikael Ebbo, Julie Graveleau, Mathieu Groh, David Launay, Sylvain Audia, Gregory Pugnet, Fleur Cohen, Antoinette Perlat, Audrey Benyamine, Boris Bienvenu, Lea Gaigne, Pascal Chanez, Jean Yves Gaubert, Nicolas Schleinitz
        European Respiratory Review Dec 2021, 30 (162) 210078; DOI: 10.1183/16000617.0078-2021
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