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An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features

Aryeh Fischer, Katerina M. Antoniou, Kevin K. Brown, Jacques Cadranel, Tamera J. Corte, Roland M. du Bois, Joyce S. Lee, Kevin O. Leslie, David A. Lynch, Eric L. Matteson, Marta Mosca, Imre Noth, Luca Richeldi, Mary E. Strek, Jeffrey J. Swigris, Athol U. Wells, Sterling G. West, Harold R. Collard, Vincent Cottin on behalf of the “ERS/ATS Task Force on Undifferentiated Forms of CTD-ILD”
European Respiratory Journal 2015 46: 976-987; DOI: 10.1183/13993003.00150-2015
Aryeh Fischer
1Dept of Medicine, University of Colorado School of Medicine, Denver, CO, USA
17Task force chair
18Members of the writing group of the task force
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  • For correspondence: aryeh.fischer@ucdenver.edu
Katerina M. Antoniou
2Thoracic Medicine, University of Crete, Heraklion, Greece
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Kevin K. Brown
3Dept of Medicine, National Jewish Health, Denver, CO, USA
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Jacques Cadranel
4Pneumologie, Hopital Tenon, Paris, France
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Tamera J. Corte
5The Aldred Hospital, Melbourne, Australia
18Members of the writing group of the task force
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Roland M. du Bois
6Interstitial Lung Disease Unit, Dept of Occupational Medicine, Royal Brompton Hospital, London, UK
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Joyce S. Lee
7Medicine, University of California San Francisco, San Francisco, CA, USA
18Members of the writing group of the task force
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Kevin O. Leslie
8Pathology, Mayo Clinic, Scottsdale, AZ, USA
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David A. Lynch
9Dept of Radiology, National Jewish Health, Denver, CO, USA
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Eric L. Matteson
10Division of Rheumatology, Mayo College of Medicine, Rochester, MN, USA
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Marta Mosca
11University of Pisa, Pisa, Italy
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Imre Noth
12Medicine, University of Chicago, Chicago, IL, USA
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Luca Richeldi
13Southampton General Hospital, Southampton, UK
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Mary E. Strek
12Medicine, University of Chicago, Chicago, IL, USA
18Members of the writing group of the task force
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Jeffrey J. Swigris
3Dept of Medicine, National Jewish Health, Denver, CO, USA
18Members of the writing group of the task force
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Athol U. Wells
14Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK
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Sterling G. West
15University of Colorado School of Medicine, Aurora, CO, USA
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Harold R. Collard
7Medicine, University of California San Francisco, San Francisco, CA, USA
18Members of the writing group of the task force
19Task force vice-chairs, and contributed equally to this manuscript
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Vincent Cottin
16Service de Pneumologie, Hopital L. Pradel, Lyon, France
18Members of the writing group of the task force
19Task force vice-chairs, and contributed equally to this manuscript
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  • Article
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Figures

  • Tables
  • Additional Files
  • FIGURE 1
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    FIGURE 1

    “Mechanic hands” characterised by distal digital fissuring and cracking of the skin.

  • FIGURE 2
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    FIGURE 2

    Gottron's sign characterised by fixed erythematous rash over the meta-carpal phalangeal joints. Note also the presence of periungual erythema.

  • FIGURE 3
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    FIGURE 3

    Bibasilar reticulation and traction bronchiectasis with minimal ground glass opacifications consistent with fibrotic non-specific interstitial pneumonia. Note also the presence of a dilated fluid-filled oesophagus.

  • FIGURE 4
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    FIGURE 4

    High-resolution computed tomography image suggesting non-specific interstitial pneumonia with organising pneumonia.

  • FIGURE 5
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    FIGURE 5

    High-resolution computed tomography image suggesting lymphocytic interstitial pneumonia. Note the extensive peribronchovascular cysts.

  • FIGURE 6
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    FIGURE 6

    Photomicrograph of histopathology slide demonstrating fibrotic non-specific interstitial pneumonia pattern. Note the uniform thickening of all of the alveolar walls and scant chronic inflammation.

  • FIGURE 7
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    FIGURE 7

    Photomicrograph of histopathology slide demonstrating lymphocytic interstitial pneumonia pattern.

  • FIGURE 8
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    FIGURE 8

    Photomicrograph of histopathology slide demonstrating follicular bronchiolitis with background pattern of non-specific interstitial pneumonia pattern. Note the large reactive lymphoid follicles (F) surrounding a bronchiole (BR).

  • FIGURE 9
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    FIGURE 9

    Photomicrograph of histopathology slide demonstrating chronic pleuritis overlying cellular non-specific interstitial pneumonia pattern.

Tables

  • Figures
  • Additional Files
  • TABLE 1

    Classification criteria for “interstitial pneumonia with autoimmune features”

    1. Presence of an interstitial pneumonia (by HRCT or surgical lung biopsy) and,

    2. Exclusion of alternative aetiologies and,

    3. Does not meet criteria of a defined connective tissue disease and,

    4. At least one feature from at least two of these domains:

      • A. Clinical domain

      • B. Serologic domain

      • C. Morphologic domain

    • A. Clinical domain

      1. Distal digital fissuring (i.e. “mechanic hands”)

      2. Distal digital tip ulceration

      3. Inflammatory arthritis or polyarticular morning joint stiffness ≥60 min

      4. Palmar telangiectasia

      5. Raynaud's phenomenon

      6. Unexplained digital oedema

      7. Unexplained fixed rash on the digital extensor surfaces (Gottron's sign)

    • B. Serologic domain

      1. ANA ≥1:320 titre, diffuse, speckled, homogeneous patterns or

        • a. ANA nucleolar pattern (any titre) or

        • b. ANA centromere pattern (any titre)

      2. Rheumatoid factor ≥2× upper limit of normal

      3. Anti-CCP

      4. Anti-dsDNA

      5. Anti-Ro (SS-A)

      6. Anti-La (SS-B)

      7. Anti-ribonucleoprotein

      8. Anti-Smith

      9. Anti-topoisomerase (Scl-70)

      10. Anti-tRNA synthetase (e.g. Jo-1, PL-7, PL-12; others are: EJ, OJ, KS, Zo, tRS)

      11. Anti-PM-Scl

      12. Anti-MDA-5

    • C. Morphologic domain

      1. Suggestive radiology patterns by HRCT (see text for descriptions):

        • a. NSIP

        • b. OP

        • c. NSIP with OP overlap

        • d. LIP

      2. Histopathology patterns or features by surgical lung biopsy:

        • a. NSIP

        • b. OP

        • c. NSIP with OP overlap

        • d. LIP

        • e. Interstitial lymphoid aggregates with germinal centres

        • f. Diffuse lymphoplasmacytic infiltration (with or without lymphoid follicles)

      3. Multi-compartment involvement (in addition to interstitial pneumonia):

        • a. Unexplained pleural effusion or thickening

        • b. Unexplained pericardial effusion or thickening

        • c. Unexplained intrinsic airways disease# (by PFT, imaging or pathology)

        • d. Unexplained pulmonary vasculopathy

    • HRCT: high-resolution computed tomography; ANA: antinuclear antibody; NSIP: non-specific interstitial pneumonia; OP: organising pneumonia; LIP: lymphoid interstitial pneumonia; PFT: pulmonary function testing. #: includes airflow obstruction, bronchiolitis or bronchiectasis.

Additional Files

  • Figures
  • Tables
  • Disclosures

    • A. Fischer
    • K.K. Brown
    • J.S. Lee
    • D.A. Lynch
    • L. Richeldi
    • M.E. Strek
    • H.R. Collard
  • Supplementary material

    Please note: supplementary material is not edited by the Editorial Office, and is uploaded as it has been supplied by the author.

    • Supplementary figure S1
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An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features
Aryeh Fischer, Katerina M. Antoniou, Kevin K. Brown, Jacques Cadranel, Tamera J. Corte, Roland M. du Bois, Joyce S. Lee, Kevin O. Leslie, David A. Lynch, Eric L. Matteson, Marta Mosca, Imre Noth, Luca Richeldi, Mary E. Strek, Jeffrey J. Swigris, Athol U. Wells, Sterling G. West, Harold R. Collard, Vincent Cottin
European Respiratory Journal Oct 2015, 46 (4) 976-987; DOI: 10.1183/13993003.00150-2015

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An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features
Aryeh Fischer, Katerina M. Antoniou, Kevin K. Brown, Jacques Cadranel, Tamera J. Corte, Roland M. du Bois, Joyce S. Lee, Kevin O. Leslie, David A. Lynch, Eric L. Matteson, Marta Mosca, Imre Noth, Luca Richeldi, Mary E. Strek, Jeffrey J. Swigris, Athol U. Wells, Sterling G. West, Harold R. Collard, Vincent Cottin
European Respiratory Journal Oct 2015, 46 (4) 976-987; DOI: 10.1183/13993003.00150-2015
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